产品货号 :  mlR2047

英文名称 :  CPT1A

中文名称 :  肉毒碱棕榈酰基转移酶1A抗体

别    名 :  CPT 1; CPT1A; CPTI-L; CPT1-L; carnitine palmitoyl transferase 1; Carnitine O-palmitoyltransferase 1, liver isoform; CPT I; Carnitine palmitoyltransferase 1A; carnitine O-palmitoyltransferase 1, liver isoform isoform 1; CPT1A_HUMAN.  

研究领域 :  肿瘤  免疫学  信号转导  激酶和磷酸酶  线粒体  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应  :  Human, Mouse, Rat,

产品应用 :  WB=1:500-2000 ELISA=1:500-1000  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.

分 子 量 :  86kDa

细胞定位 :  细胞浆 线粒体

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human CPT1A:1-100/773

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.

PubMed :  PubMed

产品介绍 :   The mitochondrial oxidation of long-chain fatty acids is nitiated by the sequential action of carnitine palmitoyltransferase I (which is located in the outer membrane and is detergent-labile) and carnitine palmitoyltransferase II (which is located in the inner membrane and is detergent-stable), together with a carnitine-acylcarnitine translocase. CPT I is the key enzyme in the carnitine-dependent transport across the mitochondrial inner membrane and its deficiency results in a decreased rate of fatty acid beta-oxidation. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008].

Function:

Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Plays an important role in triglyceride metabolism.

Subunit:

Homohexamer and homotrimer. Identified in a complex that contains at least CPT1A, ACSL1 and VDAC1. Also identified in complexes with ACSL1 and VDAC2 and VDAC3.

Subcellular Location:

Mitochondrion outer membrane; Multi-pass membrane protein.

Tissue Specificity:

Strong expression in kidney and heart, and lower in liver and skeletal muscle.

DISEASE:

Carnitine palmitoyltransferase 1A deficiency (CPT1AD) [MIM:255120]: Rare autosomal recessive metabolic disorder of long-chain fatty acid oxidation characterized by severe episodes of hypoketotic hypoglycemia usually occurring after fasting or illness. Onset is in infancy or early childhood. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:

Belongs to the carnitine/choline acetyltransferase family.

SWISS:

P50416

Gene ID:

1374

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.