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肉毒堿棕櫚酰基轉移酶1A抗體

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中文名稱 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.  

 

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 Human, Mouse, Rat, 
產品應用 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
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CPT1A:1-100/773 
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 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMed PubMed
產品介紹 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

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.
 
產品圖片 Sample:
Lane 1: Kidney (Mouse) Lysate at 40 ug
Lane 2: Kidney (Rat) Lysate at 40 ug
Lane 3: Cerebrum (Mouse) Lysate at 40 ug
Primary: Anti- CPT1A (bs-2047R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 86 kD
Observed band size: 86 kD
Sample:
Lane 1:Kidney (Mouse) Lysate at 40 ug
Lane 2: Heart (Mouse) Lysate at 40 ug
Lane 3: Liver (Mouse) Lysate at 40 ug
Lane 4: Cerebrum (Mouse) Lysate at 40 ug
Primary: Anti- CPT1A (bs-2047R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 86 kD
Observed band size: 86 kD
Sample:
Spinal cord (Mouse) Lysate at 40 ug
Primary: Anti-CPT1A (bs-2047R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 86 kD
Observed band size: 86 kD
Sample:
Kidney (Mouse) Lysate at 40 ug
Primary: Anti- CPT1A (bs-2047R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 86 kD
Observed band size: 86 kD
Sample:
Cerebrum (Mouse) Lysate at 40 ug
Primary: Anti-CPT1A (bs-2047R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 86 kD
Observed band size: 86 kD
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