Rabbit Anti-DNAJC19/FITC Conjugated antibody
|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.|
|规格价格||100ul/2980元 购买 大包装/询价|
|说 明 书||100ul|
|研究领域||心血管 细胞生物 神经生物学 信号转导|
|交叉反应||Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,|
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|
|免 疫 原||KLH conjugated synthetic peptide derived from human DNAJC19|
|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.|
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]
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.
Mitochondrion inner membrane.
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.
Belongs to the TIM14 family.
Contains 1 J domain.
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
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
该基因编码的蛋白被认为是ATP依赖性转运肽蛋白从内细胞膜转运到线粒体基质的复合物的一部分。该基因的缺陷是3-甲基谷氨酸酸尿症5型（MGA5），也称为扩张型心肌病伴共济失调（DCMA）的原因。该基因的选择性剪接导致多个转录变体。在1, 2, 6、10, 14和19号染色体上已鉴定出相关的假基因。[ RefSeq，2012月1日]