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Rabbit Anti-DNAJC19/Gold Conjugated antibody (bs-14385R-Gold)
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-14385R-Gold
英文名稱 Rabbit Anti-DNAJC19/Gold Conjugated antibody
中文名稱 膠體金標(biāo)記的DNAJC19蛋白抗體
別    名 DnaJ (Hsp40) homolog, subfamily C, member 19; DnaJ homolog subfamily C member 19; DNAJC19; homolog of yeast TIM14; Mitochondrial import inner membrane translocase subunit TIM14; PAM18; TIM 14; TIM14; TIM14_HUMAN; TIMM 14; TIMM14; translocase of the inner mitochondrial membrane 14; Translocase of the inner mitochondrial membrane 14, yeast homolog of.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul(10nm  15nm  35nm
研究領(lǐng)域 心血管  細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 12kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DNAJC19
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產(chǎn)品介紹 background:
The protein encoded by this gene is thought to be part of a complex involved in the ATP-dependent transport of transit peptide-containing proteins from the inner cell membrane to the mitochondrial matrix. Defects in this gene are a cause of 3-methylglutaconic aciduria type 5 (MGA5), also known as dilated cardiomyopathy with ataxia (DCMA). Alternative splicing of this gene results in multiple transcript variants. Related pseudogenes have been identified on chromosomes 1, 2, 6, 10, 14 and 19. [provided by RefSeq, Jan 2012]

Function:
Probable component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity.

Subcellular Location:
Mitochondrion inner membrane.

Tissue Specificity:
Ubiquitously expressed.

DISEASE:
Defects in DNAJC19 are the cause of 3-methylglutaconic aciduria type 5 (MGA5) [MIM:610198]; also known as dilated cardiomyopathy with ataxia (DCMA). MGA5 is an autosomal recessive disorder characterized by early-onset dilated cardiomyopathy, growth failure, cerebellar ataxia causing significant motor delays, testicular dysgenesis, growth failure, and significant increases in urine organic acids, particularly 3-methylglutaconic acid and 3-methylglutaric acid.

Similarity:
Belongs to the TIM14 family.
Contains 1 J domain.

Database links:

Entrez Gene: 131118 Human

Entrez Gene: 100503724 Mouse

Entrez Gene: 67713 Mouse

Omim: 608977 Human

SwissProt: Q96DA6 Human

SwissProt: Q9CQV7 Mouse

Unigene: 230601 Human

Unigene: 274266 Mouse

Unigene: 389927 Mouse



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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