• Thumbnail for Carnitine palmitoyltransferase I
    Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase (CCAT), or palmitoylCoA transferase...
    19 KB (2,459 words) - 01:45, 1 May 2024
  • Thumbnail for Carnitine palmitoyltransferase I deficiency
    Carnitine palmitoyltransferase I deficiency is a rare metabolic disorder that prevents the body from converting certain fats called long-chain fatty acids(LCFA)...
    7 KB (597 words) - 14:52, 27 October 2023
  • Thumbnail for Carnitine palmitoyltransferase II deficiency
    Carnitine palmitoyltransferase II deficiency, sometimes shortened to CPT-II or CPT2, is an autosomal recessively inherited genetic metabolic disorder characterized...
    26 KB (3,167 words) - 04:46, 1 May 2024
  • Thumbnail for Carnitine O-palmitoyltransferase
    associated with Carnitine palmitoyltransferase I deficiency CPT1B CPT1C CPT2 – associated with carnitine palmitoyltransferase II deficiency Fatty acid degradation...
    2 KB (151 words) - 07:15, 20 October 2022
  • Thumbnail for Carnitine
    acetylcarnitine ester and being transported by carnitine palmitoyltransferase I and carnitine palmitoyltransferase II. Carnitine also plays a role in stabilizing acetyl-CoA...
    30 KB (3,278 words) - 20:35, 26 April 2024
  • Thumbnail for Carnitine-acylcarnitine translocase deficiency
    [citation needed] Primary carnitine deficiency Carnitine palmitoyltransferase I deficiency Carnitine palmitoyltransferase II deficiency Reference, Genetics...
    5 KB (398 words) - 13:42, 14 September 2023
  • Thumbnail for Carnitine palmitoyltransferase II
    fatty-acid (LCFA) oxidation disorders and carnitine palmitoyltransferase II deficiency. Carnitine palmitoyltransferase I GRCh38: Ensembl release 89: ENSG00000157184...
    9 KB (1,034 words) - 23:07, 18 January 2024
  • Thumbnail for Inborn error of lipid metabolism
    palmitoyltransferase I deficiency (CPT) - Carnitine palmitoyltransferase I Carnitine palmitoyltransferase II deficiency (CPT) - Carnitine palmitoyltransferase II Acid...
    4 KB (356 words) - 21:16, 7 December 2020
  • carnitine deficiency 277.85 Disorders of fatty acid oxidation metabolism Carnitine palmitoyltransferase I deficiency Carnitine palmitoyltransferase II...
    16 KB (1,399 words) - 17:41, 9 February 2024
  • Thumbnail for Hepatomegaly
    Hunter syndrome (Spleen affected) Zellweger's syndrome Carnitine palmitoyltransferase I deficiency Granulomatous: Sarcoidosis The mechanism of hepatomegaly...
    14 KB (1,162 words) - 00:28, 11 December 2023
  • glycoprotein syndrome type 1b Carnitine palmitoyltransferase I deficiency Carnitine-acylcarnitine translocase deficiency Chloramphenicol Chlorpromazine...
    21 KB (1,265 words) - 01:46, 22 December 2023
  • deficiency) GSD type X (Phosphoglycerate mutase deficiency) Phosphoglycerate kinase deficiency Carnitine palmitoyltransferase I deficiency Carnitine...
    9 KB (899 words) - 01:15, 30 January 2023
  • including glutaric aciduria type 1 in the Oji-Cree and carnitine palmitoyltransferase I deficiency in Hutterites; these screening programs were aimed at...
    3 KB (326 words) - 19:10, 9 May 2023
  • carnitine by carnitine palmitoyltransferase I, located on the cytosolic faces of the outer and inner mitochondrial membranes. Acyl-carnitine is shuttled inside...
    32 KB (3,362 words) - 13:15, 3 May 2024
  • Thumbnail for Chromosome 11
    beta-ketothiolase deficiency beta thalassemia bipolar disorder bladder cancer breast cancer carnitine palmitoyltransferase I deficiency Charcot–Marie–Tooth...
    28 KB (1,930 words) - 22:58, 28 March 2024
  • syndrome Carnitine palmitoyl transferase deficiency Carnitine palmitoyltransferase I deficiency Carnitine palmitoyltransferase II deficiency Carnitine transporter...
    40 KB (3,648 words) - 19:46, 6 May 2024
  • Thumbnail for Malonyl-CoA decarboxylase deficiency
    as an inhibitor of the mitochondrial outer membrane enzyme carnitine palmitoyltransferase I (CPT1), which consequently inhibits fatty acid oxidation. The...
    15 KB (1,719 words) - 22:12, 20 April 2024
  • Thumbnail for Hyperammonemia
    Methylmalonic acidemia Isovaleric acidemia Propionic acidemia Carnitine palmitoyltransferase II deficiency Transient hyperammonemia of the newborn, specifically...
    12 KB (1,210 words) - 18:53, 18 March 2024
  • Thumbnail for Glibenclamide
    McGarry JD (July 1994). "More direct evidence for a malonyl-CoA-carnitine palmitoyltransferase I interaction as a key event in pancreatic beta-cell signaling"...
    14 KB (1,066 words) - 06:45, 2 March 2024
  • Thumbnail for Acyl-CoA
    oxidation.  Transport of acyl-CoA into the mitochondria requires carnitine palmitoyltransferase 1 (CPT1), which converts acyl-CoA into acylcarnitine, which...
    10 KB (1,190 words) - 10:15, 10 April 2024
  • Thumbnail for Transferase
    The deficiency is caused by mutation in the gene OXCT1. Treatments mostly rely on controlling the diet of the patient. Carnitine palmitoyltransferase II...
    60 KB (6,229 words) - 00:05, 7 March 2024
  • Thumbnail for Meldonium
    co-A is then exchanged with carnitine (via the enzyme carnitine palmitoyltransferase I) to produce a fatty acid-carnitine complex. This complex is then...
    58 KB (4,893 words) - 21:29, 10 March 2024
  • Thumbnail for Rhabdomyolysis
    phosphofructokinase deficiency, glycogen storage diseases VIII, IX, X and XI Lipid metabolism defects: carnitine palmitoyltransferase I and II deficiency, deficiency of...
    49 KB (4,816 words) - 02:56, 18 January 2024
  • Thumbnail for Triheptanoin
    Triheptanoin (category Articles with changed InChI identifier)
    metabolic diseases, such as pyruvate carboxylase deficiency and carnitine palmitoyltransferase II deficiency. It also appears to increase the efficacy of...
    13 KB (969 words) - 19:48, 13 March 2024
  • Thumbnail for Glycogen storage disease type V
    exertion. This can help distinguish McArdle's syndrome from carnitine palmitoyltransferase II deficiency (CPT-II), a lipid-based metabolic disorder which prevents...
    58 KB (6,597 words) - 05:23, 13 May 2024
  • Thumbnail for Palmitoylcarnitine
    mitochondria via the carnitine-acylcarnitine translocase. Once inside the inner mitochondrial membrane, the same Carnitine O-palmitoyltransferase family is then...
    6 KB (599 words) - 05:24, 3 January 2024
  • Thumbnail for Ketogenesis
    malonyl-CoA from acetyl-CoA. Malonyl-CoA reduces the activity of carnitine palmitoyltransferase I, an enzyme that brings fatty acids into the mitochondria for...
    19 KB (2,337 words) - 21:19, 6 March 2024
  • Thumbnail for Pirinixic acid
    free fatty acid uptake and oxidation via two enzymes: carnitine palmitoyltransferase I (M-CPT I) and medium-chain acyl-CoA dehydrogenase (MCAD). ATGL...
    7 KB (675 words) - 03:19, 6 April 2023
  • Thumbnail for CHKB (gene)
    "Structural features of the gene encoding human muscle type carnitine palmitoyltransferase I". FEBS Lett. 409 (3): 401–6. doi:10.1016/S0014-5793(97)00561-9...
    15 KB (1,756 words) - 21:55, 2 January 2024
  • Thumbnail for AMP-activated protein kinase
    carboxylase (ACC) converts acetyl-CoA to malonyl-CoA, an inhibitor of carnitine palmitoyltransferase 1 (CPT-1). CPT-1 transports fatty acids into the mitochondria...
    64 KB (7,058 words) - 23:58, 14 February 2024