DNAJC19,DNAJC19蛋白抗体

产品编号HZ-14385R
英文名称DNAJC19
中文名称DNAJC19蛋白抗体
别 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.
说 明 书0.1ml 0.2ml
研究领域心血管 细胞生物 神经生物学 信号转导
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
DNAJC19,DNAJC19蛋白抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量12kDa
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human DNAJC19
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件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.
DNAJC19,DNAJC19蛋白抗体PubMedPubMed
产品介绍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.

DNAJC19,DNAJC19蛋白抗体Similarity:
Belongs to the TIM14 family.
Contains 1 J domain.

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

DNAJC19,DNAJC19蛋白抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.