CPT1A,肉毒碱棕榈酰基转移酶1A抗体

产品编号HZ-2047R
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.
说 明 书0.1ml 0.2ml
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,
CPT1A,肉毒碱棕榈酰基转移酶1A抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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86kDa
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human CPT1A
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.
CPT1A,肉毒碱棕榈酰基转移酶1A抗体PubMedPubMed
产品介绍background:
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.

CPT1A,肉毒碱棕榈酰基转移酶1A抗体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.

Gene ID:
1374

Database links:
Entrez Gene: 1374 Human
Entrez Gene: 12894 Mouse
Entrez Gene: 25757 Rat
Omim: 600528 Human
SwissProt: P50416 Human
SwissProt: P97742 Mouse
SwissProt: P32198 Rat
Unigene: 503043 Human
Unigene: 18522 Mouse
Unigene: 2856 Rat

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