catalog number :
MBS620426
products full name :
CPT1A (Cpt1a, carnitine palmitoyltransferase 1A, liver, CPT1, CPT1-L, L-CPT1, C730027G07, carnitine palmitoyltransferase 1A, carnitine palmitoyltransferase 1, liver)
products short name :
CPT1A
products name syn :
Anti -CPT1A (Cpt1a, carnitine palmitoyltransferase 1A, liver, CPT1, CPT1-L, L-CPT1, C730027G07, carnitine palmitoyltransferase 1A, carnitine palmitoyltransferase 1, liver)
other names :
carnitine O-palmitoyltransferase 1, liver isoform isoform 1; Carnitine O-palmitoyltransferase 1, liver isoform; carnitine O-palmitoyltransferase 1, liver isoform; CPT I; CPTI-L; OTTHUMP00000237196; OTTHUMP00000237197; OTTHUMP00000237552; carnitine palmitoyltransferase I, liver; carnitine O-palmitoyltransferase I, liver isoform; carnitine palmitoyltransferase 1A (liver); Carnitine O-palmitoyltransferase I, liver isoform; CPT I; CPTI-L; Carnitine palmitoyltransferase 1A
other gene names :
CPT1A; CPT1A; CPT1; CPT1-L; L-CPT1; CPT1
uniprot entry name :
CPT1A_HUMAN
host :
Host: Goat; Source: Mouse
specificity :
Recognizes mouse CPT1A. Species sequence homology: rat, porcine.
purity :
Affinity Purified. Purified by affinity chromatography.
form :
Supplied as a liquid in Tris, pH 7.2, 0.5% BSA, 0.02% sodium azide.
storage stability :
May be stored at 4 degree C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
tested application :
ELISA (EL/EIA)
app notes :
Suitable for use in ELISA. Dilution: ELISA: 1:32000
other info1 :
Immunogen: Synthetic peptide with sequence C-PETDSHRFGKHLRQ, from the C Terminus of murine CPT1A (NP_038523.2).
products categories :
Antibodies; Abs to Enzymes, Transferase
products description :
The CPT1A gene provides instructions for making a liver enzyme called carnitine palmitoyl transferase I. This enzyme assists in the transport of certain fats, called long-chain fatty acids, into the energy-producing centers in the cell (mitochondria). This process is necessary for these fats to be broken down for energy. Long-chain fatty acids must be joined to a naturally occurring substance called carnitine for entry into the mitochondria. Carnitine palmitoyl transferase I connects the long-chain fatty acids to carnitine to promote this transport process.
ncbi acc num :
NP_001867.2
ncbi gb acc num :
NM_001876.3
ncbi mol weight :
88,368 Da
ncbi pathways :
AMPK Signaling Pathway 198868!!Adipocytokine Signaling Pathway 83093!!Adipocytokine Signaling Pathway 505!!FOXA2 And FOXA3 Transcription Factor Networks Pathway 137911!!Fatty Acid Beta Oxidation Pathway 198865!!Fatty Acid Metabolism Pathway 82935!!Fatty Acid Metabolism Pathway 296!!Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway 160977!!Import Of Palmitoyl-CoA Into The Mitochondrial Matrix Pathway 106108!!Metabolism Of Lipids And Lipoproteins Pathway 160976
ncbi summary :
The mitochondrial oxidation of long-chain fatty acids is initiated 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]
uniprot summary :
Catalytic activity: Palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine. Enzyme regulation: Inhibitors such as malonyl-CoA interact with its catalytic domain and not with an associated regulatory component. Pathway: Lipid metabolism; fatty acid beta-oxidation. Subcellular location: Mitochondrion outer membrane; Multi-pass membrane protein. Tissue specificity: Strong expression in kidney and heart, and lower in liver and skeletal muscle. Involvement in disease: Defects in CPT1A are the cause of carnitine palmitoyltransferase 1A deficiency (CPT1AD) [. MIM:255120]; also known as CPT-I deficiency or CPT1A deficiency. CPT1AD is a 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. Ref.2 Ref.6 Ref.7 Ref.8 Ref.9 Ref.10 Ref.11 Ref.12. Sequence similarities: Belongs to the carnitine/choline acetyltransferase family.