Karnitin palmitoiltransferaza I

Karnitinska palmitoiltransferaza 1A (jetra)
Identifikatori
Simbol CPT1A
Alt. simboli CPT1
Entrez 1374
Hugo 2328
OMIM 600528
RefSek NM_001876
UniProt P50416
Drugi podaci
EC broj 2.3.1.21
Lokus Hrom. 11 q13.1-13.5
Karnitinska palmitoiltransferaza 1B (mišići)
Identifikatori
Simbol CPT1B
Entrez 1375
Hugo 2329
OMIM 601987
RefSek NM_152245
UniProt Q92523
Drugi podaci
Lokus Hrom. 22 q13.3
Karnitinska palmitoiltransferaza 1C (mozak)
Identifikatori
Simbol CPT1C
Entrez 126129
Hugo 18540
OMIM 608846
RefSek NM_152359
UniProt Q8TCG5
Drugi podaci
Lokus Hrom. 19 q13.33

Karnitinska palmitoiltransferaza I (CPT1, Karnitinska palmitoiltransferaza I, CAT1) mitohondrijski je enzim.[1][2] Ona pripada familiji enzima koji se nazivaju karnitinske aciltransferaze.[3] Sledeće CPT1 izoforme su poznate: CPT1A, CPT1B, i CPT1C. CPT1 je vezana za spoljašnju mitohondrijsku membranu i posreduje transport dugolančanih masnih kiselina kroz membranu putem njihovog vezivanja za karnitin. Ova enzim se može inhibirati dejstvom malonil KoA. Njegova uloga u metabolizmu masnih kiselina ga čini značajnim za mnoge metaboličke poremećaje, npr. dijabetes. Njegova kristalna struktura nije poznata, te detaljan mehanizam njegovog dejstva nije utvrđen.

Reference

  1. ↑ van der Leij FR, Huijkman NC, Boomsma C, Kuipers JR, Bartelds B (2000). „Genomics of the human carnitine acyltransferase genes”. Mol. Genet. Metab. 71 (1–2): 139–53. DOI:10.1006/mgme.2000.3055. PMID 11001805. 
  2. ↑ Bonnefont JP, Djouadi F, Prip-Buus C, Gobin S, Munnich A, Bastin J (2004). „Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects”. Mol. Aspects Med. 25 (5–6): 495–520. DOI:10.1016/j.mam.2004.06.004. PMID 15363638. 
  3. ↑ Tong L, Gerwald J (2003). „Crystal Structure of Carnitine Acetyltransferase and Implications for the Catalytic Mechanism and Fatty Acid Transport”. Cell 112 (1): 113–122. DOI:10.1016/S0092-8674(02)01228-X. PMID 12526798. 

Literatura

  • Nicholas C. Price, Lewis Stevens (1999). Fundamentals of Enzymology: The Cell and Molecular Biology of Catalytic Proteins (Third izd.). USA: Oxford University Press. ISBN 019850229X. 
  • Eric J. Toone (2006). Advances in Enzymology and Related Areas of Molecular Biology, Protein Evolution (Volume 75 izd.). Wiley-Interscience. ISBN 0471205036. 
  • Branden C, Tooze J.. Introduction to Protein Structure. New York, NY: Garland Publishing. ISBN: 0-8153-2305-0. 
  • Irwin H. Segel. Enzyme Kinetics: Behavior and Analysis of Rapid Equilibrium and Steady-State Enzyme Systems (Book 44 izd.). Wiley Classics Library. ISBN 0471303097. 
  • Robert A. Copeland (2013). Evaluation of Enzyme Inhibitors in Drug Discovery: A Guide for Medicinal Chemists and Pharmacologists (2nd izd.). Wiley-Interscience. ISBN 111848813X. 
  • Gerhard Michal, Dietmar Schomburg (2012). Biochemical Pathways: An Atlas of Biochemistry and Molecular Biology (2nd izd.). Wiley. ISBN 0470146842. 

Vidi još

Spoljašnje veze

  • GeneReviews/NCBI/NIH/UW entry on Carnitine Palmitoyltransferase 1A Deficiency
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2.3.1: osim amino-acilne grupe2.3.2: Aminoaciltransferaze2.3.3: konverzija u alkil pri transferu
B enzm: 1.1/2/3/4/5/6/7/8/10/11/13/14/15-18, 2.1/2/3/4/5/6/7/8, 2.7.10, 2.7.11-12, 3.1/2/3/4/5/6/7, 3.1.3.48, 3.4.21/22/23/24, 4.1/2/3/4/5/6, 5.1/2/3/4/99, 6.1-3/4/5-6