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Rabbit Anti-FAM134B/BF594 Conjugated antibody (bs-13136R-BF594)
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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-13136R-BF594
英文名稱 Rabbit Anti-FAM134B/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的FAM134B蛋白抗體
別    名 F134B_HUMAN; FAM134B; family with sequence similarity 134 member B; HSAN2B; JK 1; JK1; Protein FAM134B.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse, Rat,  (predicted: Human, Cow, 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 55kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FAM134B
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:
Required for long-term survival of nociceptive and autonomic ganglion neurons.

Function:
Required for long-term survival of nociceptive and autonomic ganglion neurons.

Subcellular Location:
Golgi apparatus; cis-Golgi network membrane.

DISEASE:
Defects in FAM134B are the cause of hereditary sensory and autonomic neuropathy type 2B (HSAN2B) [MIM:613115]. A form of hereditary sensory and autonomic neuropathy, a genetically and clinically heterogeneous group of disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and by sensory and/or autonomic abnormalities. HSAN2B is an autosomal recessive disorder characterized by impairment of pain, temperature and touch sensation. Onset occurs in the first or second decade, with impaired nociception and progressive mutilating ulceration of the hands and feet with osteomyelitis and acroosteolysis. Amputations of the hands and feet are common. Autonomic dysfunction includes hyperhidrosis, urinary incontinence, and slow pupillary light response.

Similarity:
Belongs to the FAM134 family.

Database links:

Entrez Gene: 54463 Human

Entrez Gene: 66270 Mouse

Entrez Gene: 619558 Rat

Omim: 613114 Human

SwissProt: Q9H6L5 Human

SwissProt: Q8VE91 Mouse

SwissProt: Q5FVM3 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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