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carnitine palmitoyltransferase 1A (liver)
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]
Normal tissues
|
|
||
|---|---|---|---|
| Cecum |
100
|
||
| Vomeronasal organ |
94
|
||
| Stomach |
92
|
||
| Trigeminal ganglion |
89
|
||
| Dorsal striatum |
89
|
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| Preoptic area |
89
|
Compounds
|
|
||
|---|---|---|---|
| Levocarnitine |
100
|
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| Perhexilene |
100
|
||
| Dalzic |
100
|
||
| Adenylic Acid |
97
|
||
| Guanethidine |
96
|
||
| Azithromycin |
81
|
Diseases
|
|
||
|---|---|---|---|
| Carnitine palmitoylt… |
100
|
||
| High fat diet |
59
|
||
| Food deprivation |
53
|
||
| Diabetic neuropathy |
46
|
||
| Malignant fibrous hi… |
43
|
||
| Hyperglycemia |
42
|
 Individual Studies |
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|---|---|
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Mus musculus
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Mutations/Phenotypic
Homo sapiens
|
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Inhibition of hypothalamic carnitine palmitoyltransferase-1 decreases food intake and glucose production.
Authors: Silvana Obici, Zhaohui Feng, Arduino Arduini, Roberto Conti, Luciano Rossetti
Nature medicine 2003 Jun -
Overexpression of carnitine palmitoyltransferase-1 in skeletal muscle is sufficient to enhance fatty acid oxidation and improve high-fat diet-induced insulin resistance.
Authors: Clinton R Bruce, Andrew J Hoy, Nigel Turner, Matthew J Watt, Tamara L Allen, Kevin Carpenter, Gregory J Cooney, Mark A Febbraio, Edward W Kraegen
Diabetes 2009 Mar
Thought leaders and organizations working on research involving CPT1A.
- Oregon Health and Science University
- Assistance Publique - Hôpitaux de Paris
- University of Barcelona
- University of Tennessee Health Science Center
- Albert Einstein College of Medicine
- Baker IDI Heart and Diabetes Institute
- University of Illinois at Chicago
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Metabolic Consequences of CPT-1 Deficiency
conditions: Carnitine Palmitoyl Transferase Type 1A (CPT1A) Deficiency
interventions: Medically supervised fasting
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Nutritional Therapy of the Deficits of Oxidation Mitochondrial of the Fatty Acids
conditions: Inborn Errors of Metabolism
interventions: Oil special 107 and MYGLIOL 810
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