CPT1A Chromosome 11

Carnitine palmitoyltransferase 1A
175 variants 175 Health Risk

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What This Gene Does
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, Jul 2008]
Gene Info
Gene Group
Carnitine palmitoyltransferases
Locus Type
gene with protein product
Location
11q13.3
Ensembl
ENSG00000110090
Associated Conditions (13)
Carnitine palmitoyl transferase 1A deficiency
CPT1A-related disorder
Inborn genetic diseases
Malignant tumor of esophagus
Nonpapillary renal cell carcinoma
Sarcoma
Thyroid cancer
nonmedullary
1
Clear cell carcinoma of kidney
Ovarian serous cystadenocarcinoma
CPT1A ARCTIC VARIANT
CPT1A POLYMORPHISM
Key Variants
RS114030714
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, CPT1A-related disorder, Carnitine palmitoyl transferase 1A deficiency
Health Risk
RS1165511243
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
Health Risk
RS139789100
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
Health Risk
RS141191480
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
Health Risk
RS141658962
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
Health Risk
RS147389938
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, CPT1A-related disorder
Health Risk
RS150792109
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, CPT1A-related disorder, Malignant tumor of esophagus
Health Risk
RS151271754
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, CPT1A-related disorder, Carnitine palmitoyl transferase 1A deficiency
Health Risk
RS1855050882
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
Health Risk
RS199640034
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
Health Risk
RS200893871
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
Health Risk
RS201425412
Conflicting classifications of pathogenicity
Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
Health Risk
All Variants (175)
RSID Category Clinical Significance Conditions
RS114030714 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, CPT1A-related disorder, Carnitine palmitoyl transferase 1A deficiency
RS1165511243 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
RS139789100 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS141191480 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
RS141658962 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS147389938 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, CPT1A-related disorder
RS150792109 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, CPT1A-related disorder, Malignant tumor of esophagus
RS151271754 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, CPT1A-related disorder, Carnitine palmitoyl transferase 1A deficiency
RS1855050882 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS199640034 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS200893871 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
RS201425412 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
RS373015421 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
RS398123653 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, CPT1A-related disorder, Carnitine palmitoyl transferase 1A deficiency
RS542856213 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
RS552007692 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS569816517 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS751235722 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
RS755308448 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS757593086 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
RS766819782 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS767840321 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS772868108 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Inborn genetic diseases, Carnitine palmitoyl transferase 1A deficiency
RS77477448 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, CPT1A-related disorder, Inborn genetic diseases
RS778920950 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS80356775 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS80356783 Health Risk Conflicting classifications of pathogenicity Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1057516304 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1057516396 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1057516434 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1057516586 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1057517046 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1057517188 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1057517245 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS112498048 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1189381456 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1193442541 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1212529394 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1224226554 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1269472669 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1282293820 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1293504009 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1401811144 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1427012032 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1463640534 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1555226417 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1555227607 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1555228470 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1555228640 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
RS1555230326 Health Risk Likely pathogenic Carnitine palmitoyl transferase 1A deficiency, Carnitine palmitoyl transferase 1A deficiency
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