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Rabbit Anti-CPT1A/BF594 Conjugated antibody (bs-2047R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-2047R-BF594
英文名稱1 Rabbit Anti-CPT1A/BF594 Conjugated antibody
中文名稱 BF594標記的肉毒堿棕櫚酰基轉移酶1A抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  免疫學  信號轉導  激酶和磷酸酶  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, )
產品應用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 86kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CPT1A
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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.

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.

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.
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