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Rabbit Anti-Myocilin/BF647 Conjugated antibody (bs-19176R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-19176R-BF647
英文名稱 Rabbit Anti-Myocilin/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的開角型青光眼Myocilin抗體
別    名 Glaucoma 1 open angle; Glaucoma 1 open angle; GLC1A; GPOA; JOAG; JOAG1; Mutated trabecular meshwork-induced glucocorticoid response protein; MYOC; MYOC_HUMAN; Myocilin; Myocilin, trabecular meshwork inducible glucocorticoid; TIGR; Trabecular meshwork induced glucocorticoid response protein; Trabecular meshwork-induced glucocorticoid response protein.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Rabbit, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 53kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Myocilin
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
MYOC encodes the protein myocilin, which is believed to have a role in cytoskeletal function. MYOC is expressed in many occular tissues, including the trabecular meshwork, and was revealed to be the trabecular meshwork glucocorticoid-inducible response protein (TIGR). The trabecular meshwork is a specialized eye tissue essential in regulating intraocular pressure, and mutations in MYOC have been identified as the cause of hereditary juvenile-onset open-angle glaucoma. [provided by RefSeq, Jul 2008]

Function:
May participate in the obstruction of fluid outflow in the trabecular meshwork.

Subcellular Location:
Rough endoplasmic reticulum. Secreted. Cell projection > cilium. Located preferentially in the ciliary rootlet and basal body of the connecting cilium of photoreceptor cells, and in the rough endoplasmic reticulum. Also secreted.

Tissue Specificity:
Expressed in large amounts in various types of muscle, ciliary body, papillary sphincter, skeletal muscle, heart and other tissues. Expressed predominantly in the retina. In normal eyes, found in the inner uveal meshwork region and the anterior portion of the meshwork. In contrast, in many glaucomatous eyes, it is found in more regions of the meshwork and appeared more intensively than in normal eyes, regardless of the type or clinical severity of glaucoma.

Post-translational modifications:
Different isoforms may arise by post-translational modifications.
Glycosylated.
Palmitoylated.

DISEASE:
Defects in MYOC are the cause of primary open angle glaucoma type 1A (GLC1A) [MIM:137750]. Primary open angle glaucoma (POAG) is characterized by a specific pattern of optic nerve and visual field defects. The angle of the anterior chamber of the eye is open, and usually the intraocular pressure is increased. The disease is asymptomatic until the late stages, by which time significant and irreversible optic nerve damage has already taken place. Defects in MYOC may also contribute to primary congenital glaucoma type 3A (GLC3A) [MIM:231300].
Defects in MYOC may contribute to this phenotype via digenic inheritance. GLC3A is an autosomal recessive form of primary congenital glaucoma (PCG). PCG is characterized by marked increase of intraocular pressure at birth or early choldhood, large ocular globes (buphthalmos) and corneal edema. It results from developmental defects of the trabecular meshwork and anterior chamber angle of the eye that prevent adequate drainage of aqueous humor.

Similarity:
Contains 1 olfactomedin-like domain.

Database links:

Entrez Gene: 4653 Human

Entrez Gene: 17926 Mouse

Entrez Gene: 81523 Rat

Omim: 601652 Human

SwissProt: Q99972 Human

SwissProt: O70624 Mouse

SwissProt: Q9R1J4 Rat

Unigene: 436037 Human

Unigene: 10694 Mouse

Unigene: 30051 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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