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DNAJC19 Rabbit pAb (bs-14385R)  
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產(chǎn)品編號 bs-14385R
英文名稱 DNAJC19 Rabbit pAb
中文名稱 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 m  
研究領域 心血管  細胞生物  神經(jīng)生物學  信號轉導  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse (predicted: Human,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse)
產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 12 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DNAJC19: 21-116/116 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
Q96DA6

Gene ID:
131118

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



產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (DNAJC19) Polyclonal Antibody, Unconjugated (bs-14385R) at 1:500 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.
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