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gene expression
genetic association
copy number variations
DNA correlation
duplication
gene recognition
1/f noise
Zipf's law
absolute pitch
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bioinformatics courses and degrees
statistical genetics courses
microarray courses
complete genomes
CV
publications
presentations
meetings/trips
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links to past
100 human genes
2005 Mexico course
on genetic association analysis
Perl code for recursive
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The purpose of web project is to collect 100 (or more?) human genes that
are basic, or important, or interesting, or getting media attention, and perhaps should
be known by a biology/genetics student. If you would like to suggest a human
gene to be added to this list, please send me a message! (Wentian Li, wli@nslij-genetics.org)
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disease
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apolipoprotein E (APOE) gene. chromosome 19. three alleles, e2, e3
(most coomon), e4. e4 is a risk allele for Alzheimer disease.
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Huntington disease gene. the first gene being mapped by
positional cloning. and the gene was found in 1993. chromosome 4 (4p16).
(detailed description in Reilly's book). 10-35 copies of CAG is normal,
36 or more become HD. the mutation leads to "gain of function.
huntingtin protein.
* [note sure]
TSC is a rare disease that causes benigh tumors to grow in brain
and other organs.
http://en.wikipedia.org/wiki/Tuberous_sclerosis
Probably too rare to include?
Tuberous Sclerosis: mutations in the TSC1 and TSC2 genes cause tuberous
sclerosis. The TSC1 and TSC2 genes provide instructions for making the
proteins hamartin and tuberin respectively. The proteins act as tumor
suppressors, which normally prevent cells from growing and dividing too
fast or in an uncontrolled way. Mutations in either the TSC1 or TSC2
gene lead to the production of a nonfunctional version of hamartin or
tuberin. As a result, cells can divide excessively to form numerous
tumors associated with tuberous sclerosis.
[jun24-08]
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Lou Gehrig disease gene: neurodegenerative disease caused by the
degeneration of motor neurons, the nerve cells in the central nervous
system that control voluntary muscle movement.
Also known as ALS (Amyotrophic Lateral Sclerosis), Maladie de Charcot
disease. 10% of ALS patients are caused by a mutation in SOD1 gene
(chr21), ?% are caused by a mutation in ALS2 gene (chr2).
The protein product of ALS2 gene is "Alsin", particularly abundant
in motor neurons.
ALS2: gene provides instructions for making a protein called alsin. It
is particularly abundant in motor neurons, the specialized nerve cells
in the brain and spinal cord that control the movement of muscles.
Alsin's function in cells is unclear. It may play a role in regulation
cell membrane organization and the movement of molecules inside cells.
Alsin may play a role in the development of axons and dendrites, which
are specialized outgrowths from nerve cells that are essential for the
transmission of nerve impulses.
Diseases:
amyotrophic lateral sclerosis- caused by mutations in the ALS2 gene;
loss of alsin protein somehow causes type 2 amyotrophic lateral
sclerosis. Loss of alsin may disrupt the movement of essential
molecules within cells or alter the development of axons and dendrites.
infantile-onset ascending hereditary spastic paralysi- caused by
mutations in the ALS2 gene; exact mechanism is unclear
juvenile primary lateral sclerosis- caused by mutations in the ALS2
gene; exact mechanism is unclear
[jun11-08]
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familial AF: rapid, irregular beating of the left atrium (upper chamber) of the heart.
uncoordinated electrical activity in the heart's upper chambers (the atria).
perhaps 30% of AF have family history. KCNQ1 - ch7.
Familial Atrial Fibrillation: mutations in the KCNQ1, genes KCNE2 and
KCNJ2 are also associated with this disease. These genes are important
for providing instructions for making proteins that act as channels
across the cell membrane. These channels transport positively charged
atoms (ions) of potassium into and out of cells.
[jun24-08]
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One of the early-onset (familial) Alzheimer's disease gene:
Amyloid precursor protein (APP). It is a large nerve-protecting
protein that is the source of beta amyloid. It was the first
discovered AD gene (in 1991). Duplication of APP leads to
some familiar AD.
APP: autosomal dominant mutations in APP cause hereditary early-onset
Alzheimer's disease, as a result of proteolytic processing of the alpha,
beta and gamma secretases. Increases in either total AB levels or the
relative concentrations of both AB40 and AB42 (where the former is more
concentrated in cerebrovascular plaques and the latter in neuritic
plaques) have been implicated in the pathogenesis of both familial and
sporadic Alzheimer's disease.
[jun11-08]
* Tay-Sachs disease: rare, and was called a Jewish disease. It's effectively
eliminated from the population by the genetic counseling.
http://en.wikipedia.org/wiki/Tay-Sachs_Disease
HEXA on chromosome 15. Probably too rare to include.
Tay-Sachs Disease: disorder that causes progressive destruction of nerve
cells in the brain and spinal cord. The HEXA gene provides instructions
for making part of an enzyme called beta-hexosaminidase A, which plays a
critical role in the central nervous system. Mutations in the HEXA gene
disrupt the activity of beta-hexosaminidase A, preventing the breakdown
of this substance. As a result, it can accumuate to toxic levels in the
brain and spinal cord.
[jun24-08]
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NF1 disease gene: Neurofibromin 1 on chromosome 17q11.2.
It is the second gene that Francis Collins identified.
(http://www.understandingnf1.org/)
NF1: involved in autosomal dominant cancer prone disease,
neurofibromatosis type 1 is an hamartoneoplastic syndrome; also involved
in Watson syndrome (autosomal dominant disease with cardiac
malformations and is found in von Recklinghausen neurofibromatosis, low
normal intelligence, cafe-au-lait spots, and neurofibromas but to a
lesser extend (atlasgeneticsoncology.org/Genes/NF1ID134.html)
[jun11-08]
evolution
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what makes human being human? (from Carroll's book) FOXP2
(see also, http://genes.uchicago.edu/lahn.html)
Nature 413:519-523 (2001)
Nature 418:869-872 (2002).
same is found in Neanderthals - maybe it was derived from
Neanderthals?
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MCPH1 regulates brain size.
cellular functions
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histone
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RNA polymerase II (actually, there is a whole family of
proteins involved in transcription: TFII B,D,E,F,H, TBP...) (2006 Nobel prize in chemistry).
http://nobelprize.org/nobel_prizes/chemistry/laureates/2006/press.html
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cell cycle cdc2 gene:
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prion: the normal function is as a copper dependent antioxidant
(slowing or preventing the oxidation of other molecules).
but in mutated form, it's not digested by protease, causing
Creutzfeldt-Jakob Disease (mad cow)
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dicer (multidomain ribonuclease that processes double-stranded RNAs
to 21-nt small interfering RNAs) [ch14], and RISC (RNA-induced sliencing complex)
(complex that binds these fragments.
http://nobelprize.org/nobel_prizes/medicine/laureates/2006/press.html
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ubiquitin (2004 Nobel chemistry)
cell regulate the breakdown of intracellular proteins. a housekeeping gene
http://nobelprize.org/nobel_prizes/chemistry/laureates/2004/press.html
Its main known function is to mark other proteins for destruction, known as proteolysis.
Ubiquitin can also mark transmembrane proteins (for example, receptors) for removal from membranes.
76 aa.
Once the "death mark" is applied, proteins are shuttled to a cell structure called a proteasome, which pulls the protein in and chops it into pieces. However, if ubiquitin hydrolase intervenes and removes the ubiquitin, the protein is saved.
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heat shock proteins (HSP70, HSP90,..). Ritossa (1962) paper.
[can also be in cancer category since hyperthermia is a cancer treatment]
development, sex, aging
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homeostic genes (homeo-box, or, HOX). ~300 (235 functional, 65
paseudo). 180 nt or 30 aa. Oct 26, 2007 issue of BMC Biology.
http://nobelprize.org/nobel_prizes/medicine/laureates/1995/press.html
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hSHH (human sonic hedgehog gene): not a transcription factor
- sex-determining genes: SRY (on Y chromosome), DAX1 (on X chromosome).
http://www.eurekalert.org/pub_releases/2003-04/nu-tg-040803.php
A gene on Y chromosome is very rare.
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SRY and maleness. turns on SOX9 and other genes
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SHC (three splice variants: p46, p52, p66). in mice, disruption of p66
(involved in programmed cell death) increase lifespan by 30%.
Src homology 2 domain containing.
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SIRT1 (yeast, mice) gene product turns off genes which make proteins
that help to store fat. it also commands liver cell to break down fat.
(Leonard Guarente of Harvard)
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TOR (target of rapamycin): another gerotogene completes with SIRT1
(Brian Kennedy, Matt Kaeberlein)
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telomerase and its potential role in aging.
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cytochrome c (cyt c, ch7): reintroduction of oxygen leads to production
of several proteins (including cyt c) which triggers apoptosis.
enzyme, digestive system
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kinase: enzyme for phosphorylation. target can be protein, sugar,
nucleotides, lipid. more than 600 kinase genes.
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DNase, RNase
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lactase (LCT), a member of the beta-galactosidase family. the E.coli
beta-gal is related to the Nobel prize of medicine in 19??.
lactose intolerance.
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hungry signals: leptin (from fat-storing cells), ghrelin
(stomach), PYY (small intistine), and insulin from pancreas
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hexokinase: the first step of glycolysis (convert glucose to ATP) is
for this kinase to phosphorylate glucose. won 1947 Nobel prize in
medicine:
http://nobelprize.org/nobel_prizes/medicine/laureates/1947/press.html
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Hypercholesterolemia (high blood cholesterol):
http://en.wikipedia.org/wiki/Hypercholesterolemia.
LDLR is the work that won 1985 Nobel Prize.
Hypercholesterolemia: mutations in the APOB, LDLR, LDLRAP1, and PCSK9
genes cause hypercholesterolemia. The most common cause is a mutation
in the LDLR gene. The LDLR gene provides instructions for making a
protein called a low-density lipoprotein receptor. Some LDLR mutations
reduce the number of low density lipoprotein receptors produced within cells.
[jun24-08]
blood, heart,
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ABO blood type gene. chromosome 9.
http://en.wikipedia.org/wiki/ABO_blood_group_system
1930 Nobel prize in medicine:
http://nobelprize.org/nobel_prizes/medicine/laureates/1930/press.html
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beta globin gene on chr11 (related to sickle cell disease).
HBG1: 5,226,089-5,227,693
HBG2: 5,230,996-5,623,595
a closely related protein is Myoglobin, whose structure won
the 1962 chemistry Nobel prize
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albumin : 60% of all plasma protein.
Albumin is essential for maintaining the osmotic pressure needed for proper distribution of body fluids between intravascular compartments and body tissues. It also acts as a plasma carrier by non-specifically binding several hydrophobic steroid hormones and as a transport protein for hemin and fatty acids.
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fibrin: involved in the clotting of blood.
Thrombin: is a coagulation protein that has many effects in the coagulation
cascade. It is a serine protease that converts soluble fibrinogen into insoluble
strands of fibrin, as well as catalyzing many other coagulation-related reactions.
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ApoA: the story of Apolipoprotein A-I_M allele in (AJHG, 37(6):1083 (1985))
a small lakeside village in northern italy (Limone sul Garda). people have this
mutation/allele don't suffer from heart attack or stroke. (Circulation, 103(15):1949-1954
(2001)). note a link to ApoE, another entry.
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erythropoietin: produced in kidney. stimulate red cell production.
anaemia.
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EPAS1: make transcription factor which stimulates the production of
red blood cell. Tibetan carry a polymorphism in this gene to
make them adapt to the low oxygen level at high altitude.
endocrine system, hormone, chemical signal, signal transduction
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growth hormone (GH). chr17.
gigantism, pituitary dwarfism
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insulin (only 51 amino acids). chr11. Sanger determined its
sequence and structure, which won him 1958 Nobel chemistry prize
http://nobelprize.org/nobel_prizes/chemistry/laureates/1958/sanger-lecture.html
the connection between insulin and diabetes mellitus won 1922 Nobel prize in
medicine:
http://nobelprize.org/nobel_prizes/medicine/laureates/1923/press.html
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glucagon: the opposite of insulin. Produced by the pancreas, it is
released when the glucose level in the blood is low
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melatonin receptor
- check more peptide hormones:
http://en.wikipedia.org/wiki/Hormone
e.g. from pituitary: ACTH, ADH, FSH, LH, TSH, PRL, OXT.
from thyroid: CT, PTH.
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oxytocin (neurotransmitter) (OXT). chr20. won Nobel prize in 1955.
http://nobelprize.org/nobel_prizes/medicine/laureates/1955/theorell-lecture.html
vasopressin and oxytocin (two hormones)
and science of love
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the neurophysiological subsystems are regulated by
two major neurotransmitters, and their receptors belong to
alpha or beta. beta is mainly related to the heart function.
alpha receptors are much broader. noradregergic/adrenergic
system is associated with survival (arapid arousal, vigilance, hostiliyu..).
alpha-2-adrenoceptor (ADRA2A) and beta-2-adrenoceptor (ADRB2)
and endurance training.
immune system
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immunoglobulins (also known as antibodies). won 1972 Nobel
price in medicine
http://nobelprize.org/nobel_prizes/medicine/laureates/1972/press.html
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NF-kB: found in almost all animal cell types and is involved in cellular
responses to stimuli such as stress, cytokines, free radicals, ultraviolet irradiation,
oxidized LDL, and bacterial or viral antigens. first discovered by David Baltimore
in 1986.
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AIDS restriction genes (Nature Genetics, 36:565-574 (2004))
(Science, 313:462-466 (2006)), (Science, 317:944-947 (2007))
the first one being reported was CCR-D32
(http://home.ncifcrf.gov/ccr/lgd/)
Humans are extraordinarily sensitive to HIV (as compared to chimp). one
protein makes us more susceptible to HIV - TRIM5(alpha).
Michael Emerman (Fred Hutchinson)
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TUMOR NECROSIS FACTOR (TNF).
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MHC or HLA gene family, chr6
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T-cell receptor, chr14.
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pattern recognition receptor:
http://en.wikipedia.org/wiki/Pattern_recognition_receptor
(1) Toll-like receptor in innate immune system
http://en.wikipedia.org/wiki/Toll-like_receptor
membrane-bound. TLR1-TLR11.
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lysozyme (in tear, airway..): the natural antibiotic
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over 20 proteins in "complement system"
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X-linked SCID (severe combined imune deficiency): IL2RG (interleukin-2 receptor, gamma)
brain and neural systems
- ASPM gene and brain size
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BDNF gene and memory. "brain food" that encourages growth of neurons
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Neurotransmitter receptor complexes, such as the N-methyl-D-aspartate receptor complex (NRC/MASC),
http://bfgp.oxfordjournals.org/cgi/content/abstract/5/1/66
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axon guide: netrins ephrins semaphorins slits
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a potassium channel. (MacKinnon)
integral membrane protein (IMP) (2003 Nobel prize in chemistry)
http://nobelprize.org/nobel_prizes/chemistry/laureates/2003/press.html
[what about Agre's water channel?]
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PMP22 (peripheral myelin protein 22) on ch17: its duplication contributes
probably 80% of the CMT disease (Jena-Martin Charcot (1825-1893),
his student Pierre Marie, and independently, ENglish physician Howard Henry Tooth).
there are two large groups I (myelin, the wrap, damage), II (axon, the center,
damage), and 18 subtypes.
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N-methyl-d-aspartate receptor NR2B subunit gene GRIN2B .
smart mouse study. from Joe Tsien: He's talking about Doogie, a mouse that
over-expresses a "smart" gene in the hippocampus, a portion of the brain
critical to memory and attacked by Alzheimer's. NMDA receptors are essentially
small pores on cell membranes that let ions in and increase neuronal activity
and communication. Younger people have higher amounts of a NMDA subunit, NR2B,
that keeps communication channels open longer so more information is shared.
Neurons are equipped with a "coincidence detector" called the NMDA receptor
that is triggered only when it receives two signals from independent sources.
When it senses such a coincidence, it responds by opening a gate in the cell membrane.
The sequence is like this:
1.The NMDA receptor is blocked by a magnesium ion.
2. Then, two signals occur simultaneously: a) neuron A emits a signal in
the form of a glutamate molecule, which binds to the NMDA receptor on neuron B;
and b) the cell membrane of neuron B undergoes a reversal of electrical charge,
called depolarization.
3. When both these steps occur, the magnesium ion gets kicked out of the NMDA gate,
the channel opens, and calcium ions start flowing into the cell.
This initiates a chain of events that leads to learning.
As people age, they switch to subunit NR2A, presumably because evolution has
figured out by then we should have transmitted our genes to offspring, he says.
Dr. Tsien and his colleagues made Doogie by over-expressing the NR2B gene and
a conditional knockout by eliminating another NMDA receptor subunit.
structures
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collagen: (like a rope) main protein of connective tissue in animals and
the most abundant protein
in mammals, making up about 25% of the whole-body protein content.
it's long fiber like protein, unlike the globular protein such as enzymes.
bundles of collagen are main component of
extracellular matrix. elastin :?
COL4A3 = collagen, type IV, alpha 3
This gene carries the instructions for making one component of type IV
collagen, which is a flexible protein that forms complex networks.
Specifically, this gene makes the alpha3(IV) chain of type IV collagen.
Diseases:
Alport syndrome- caused by mutations in the COL4A3 gene. The autosomal
recessive form of Alport syndrome resutls when two copies of the COL4A3
gene in each cell are mutated.
Thin basement membrane nephropathy is an autosomal dominant condition.
Goodpasture syndrome is a severe disease of the lungs and kidneys caused
by antibodies to the alpha3(IV) collagen chains.
[jun11-08]
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proteoglycan:
a special class of glycoproteins (proteins that contains
"glycans" covalently attached to their polypeptide
side-chains. swell up with water. cartilage/joint.
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ACE gene and muscle performance
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N-cadherin (cell adhesion protein)
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motor protein:
actin (a housekeeping gene), myosin (two heads) , kinesins, dynein.
[muscle -> muscle cell -> myofibrils (cylindrical) -> actin/myosin]
(alpha-actinin-3, ACTN3?, AJHG, 73:627-631 (2003). ACTN3 only
in "fast twitch" (typeII) fibers, whereas ACTN2 in all muscle fibers)
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Myostatin (formerly known as Growth differentiation factor 8),
a growth factor that limits muscle tissue growth, i.e. higher concentrations
of myostatin in the body may cause the individual to have less developed muscles.
Ref: Schuelke, NEJM, 350:2682-2688 (2004). the story of a strong German boy...
http://en.wikipedia.org/wiki/Myostatin
MYO-29 is a myostatin blocker.
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LRP5.
Richard P. Lifton's
"High Bone Density Due to a Mutation in LDL-Receptor?Related Protein 5"
(NEJM, 346:1513-1521 (2002))
http://opa.yale.edu/news/article.aspx?id=3158
5 senses
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Color blindness. Many genes involved in color vision are on
X chromosome, making color blindness more common in makes than in females
(because it's a recessive trait). But not all CB genes are on X chromosome.
"achromatopsia 2" or "total colorblindness"
(
http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=216900):
CNGA3.
"partial colorblindness": genes are on X chromsomes
Color vision deficiency: mutations in the CNGA3, CNGB3, GNAT2, OPN1LW,
OPN1MW, and OPN1SW. Genetic changes involving the OPN1LW and OPN1MW
genes cause red-green color vision defects. Changes in the CNGA3,
CNGB3, and GNAT2 genes are responsible for achromatopsia.
[jun24-08]
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olfactory receptor genes: 1000 of them (3% of all human genes!)
2004 Nobel prize in medicine.
http://nobelprize.org/nobel_prizes/medicine/laureates/2004/press.html
G protein -> cyclic-AMP -> ion channels.
odorant receptors belong to the Gprotein coupled receptors (GPCR).
each receptor goes through cell membrane 7 times.
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alpha- beta- endorphin (pain reducer). examples of "opiod peptide".
acupencture to release endorphin.
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eye color genes: EYCL1 (green/blue), EYCL2 (brown), EYCL3 (brown/blue).
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deafness genes. cochlea is the head of the snail in inner ear. 100 genes.
connexin 26 (GJB2: gap junction protein beta-2) ch13 is responsible for more.
special genomic feature
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longest protein, titin (33,000 amino acids), on chr2.
longest gene, coding muscle protein dystrophin (2.4Mb, 3500
amino acids). the gene is so long that it takes 16 hours
to transcribe. Actually it is also a disease gene:
lack of it leads to muscular dystrophy, and a mutation
leads to Duchenne muscular dystrophy
non-protein-coding genes (i.e. RNA-coding genes)
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ribosome RNA gene:
http://sandwalk.blogspot.com/2008/01/human-ribosomal-rna-genes.html
polymorphism in copy number of rRNA genes:
http://www.genome.org/cgi/content/abstract/18/1/13.
4 kinds: 18S (making small subunit of robosome), 28S/5.8S/5S (making
large subunit)
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transfer RNA gene. 450 of them. 32 kinds of tRNA (~4S, 73-93 nt)
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micro RNA (22 nt)
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one mitochondrial gene? which one? 16500 bases with 37 genes.
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Xist (X Inactivation Specific Transcript) is an RNA gene on X chromosome.
it is expressed in the inactive X chromosome.
The inactive X chromosome is coated with this RNA.
appearence
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Melanocortin receptors. MCR1 (ch16): 80% human red hair
or fair skin have a dysfunctional variant of the MCR1 gene.
(Valverde, Healy, Jackson, Rees, Thody (1995), "Variants of the
melanocyte-stimulating hormone receptor gene are associated with red hair
and fair skin in humans", Nature Genetics, 11(3):328-330.)
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eye lenz: crystallin gene. The soluble proteins (crystallins)
that constitute the bulk of the cellular, transparent
eye lens are encoded by a surprisingly diverse group of genes.
alpha (part of heatshock),
beta, gamma (calcium binding),
epsilon (lactate dehydrogenase).
recruited by lenz because of their stable structure
(Wistow, Piatigorsky).
stem cell
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Shina Tamanaka of Kyoto Univ (nov 2007) reprogrammed adult human skin cells to
pluripotent stem cells, by using these 4 regulatory genes:
oct3/4, sox2, Klf2, c-myc.
cancer
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SRC: a proto-oncogenic tyrosine kinases.
won 1989 Nobel prize in medicine.
http://nobelprize.org/nobel_prizes/medicine/laureates/1989/press.html
SH2, SH3 domains are SRC-homologous
Src Protein-
signaling protein that specializes in messages that
control the growth of cells. It is a tyrosine kinase, so it relays its
messages by adding phosphate groups to special tyrosine amino acids in
protein chains. It adds phosphate groups to a wide variety of proteins
that control cellular structure, cell communication, and cellular
growth, turning them "on" and releasing them to perform their individual tasks.
[aug13-08]
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oncogene: RAS, WNT, MYC (transcription factor), ERK
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tumor suppressor gene, e.g., p53 (also TP53)
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BRCA1: breast cancer gene, related to repairing damaged DNA.
chromosome 17.
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EGFR: epidermal growth factor receptor. overexpression of this gene leads
to cancen including Glioblastoma.
time and rhythm
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circadian genes: PER, CRY (also PER2, PER3).
clock genes - in animals, the circadian behavior is analyzed as an
integrated system, beginning with genes leading ultimately to behavioral
outputs. The molecular mechanism of circadian clocks has been uncovered
by the use of phenotype-driven genetic analysis in a number of model
systems. The capacity for circadian gene expression is widespread
throughout the body in mammals.
[aug13-08]
not yet classified
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P57(KIP2) aka CDKN1C (cyclin-dependent kinase inhibitor 1C)
a disease gene for Beckwith-Wiedemann syndrome (an overgrowth
symdrome), might also be a risk gene for breast cancer.
located on ch11p5. p57 is tumor suppressor, part of cip/kip
gene family.
CDKN1C: protein encoded by this gene is a tight-binding, strong
inhibitor of several G1 cyclin/Cdk complexes and a negative regulator of
cell proliferation. Mutations in this gene are implicated in sporadic
cancers and Beckwith-Wiedemann syndrome, suggesting that this gene is a
tumor suppressor candidate.
[jun11-08]
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Leukoencephalopathy with vanishing white matter is an autosomal
recessive disorder characterized by a chronic and progressive course
with additional episodes of rapid deterioration provoked by fever and
minor head trauma.
Eukaryotic translation initiation factors:
In some patients, this disorder was mapped to 3q27
and was caused by mutations in the EIF2B5 gene, which encodes the
epsilon subunit of a eukaryotic translation initiation factor, EIF2B and
maps to 14q24. In a mutation study for patients who had mutations in
neither EIF2B5 nor EIF2B2, mutations were found in the other 3 subunits
of EIF2B, including EIF2B1.
[jun11-08]
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http://en.wikipedia.org/wiki/Marfan_syndrome
Flo Hyman (1954-1986), the tallest member of 1984 US women volleyball
team that won silver in olympic, had Marfan syndrome.
Marfan syndrome: a disorder of the connective tissue, affects most
organs and tissues, especially the skeleton, lungs, eyes, heart and the
aorta. Mutations in the FBN1 gene cause Marfan syndrome. The FBN1 gene
provides instructions for making a protein called fibrillin-1. A
mutation in the FBN1 gene can reduce the amount and/or quality of
fibrillin-1 that is available to form microfibrils. As a result, growth
factors are released inappropriately, causing the characteristic
features of Marfan syndrome. The TGFBR2 gene provides instructions for
making protein that transmits signals from the cell surface to other
signaling molecules inside the cell. Mutations in the TGFBR2 gene
altering the signaling activity of the protein, which disturbs the
growth and development of cells and tissues.
[jun24-08]
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Aconitase: iron regulatory protein 1
http://biocurious.com/molecule-of-the-month-aconitase-and-iron-regulatory-protein-1
key player in the central pathway of energy production.
As part of the tricarboxylic acid cycle, it converts citrate into
isocitrate. The cytosolic form of aconitase also acts as iron
regulatory protein 1. When iron levels in the cell get low, there isn't
enough iron to regenerate the cluster and the protein shifts to its
second function. This protein inhibits the formation of ferritin so the
cell can pick up more transferrin out of the blood, and with it, more iron.
[aug13-08]
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CREB gene and alcoholism
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G-protein (3 units, alpha, beta, gamma). G-protein-coupled receptor. harmone
binds to G-pro-coupled rec, it triggers G-alpha to leave, and G-double-phosphate
(GDP) leaves, replaced by G-triple-phosphate (GTP). this G-alpha then
triggers another enzyme, that converts ATP (A-3pho) to cyclic AMP (A-1pho).
1994 Nobel prize in medicine.
oncogeneoncogene RAS is a G-protein
http://nobelprize.org/nobel_prizes/medicine/laureates/1994/press.html
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obesity: FTO?
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