title: Analysis of plasma ghrelin in patients with medium-chain acyl-CoA dehydrogenase deficiency and glutaric aciduria type II.
abstract: OBJECTIVE
  Ghrelin requires a fatty acid modification for binding to the GH secretagogue receptor. Acylation of the Ser3 residue of ghrelin is essential for its biological activities. We hypothesized that acyl-CoA is the fatty acid substrate for ghrelin acylation. Because serum octanoyl-CoA levels are altered by fatty acid oxidation disorders, we examined circulating ghrelin levels in affected patients.
  
  
  METHODS
  Blood levels of acyl (A) and des-acyl (D) forms of ghrelin and acylcarnitine of patients with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency and glutaric aciduria type II (GA2) were measured.
  
  
  RESULTS
  Plasma acyl ghrelin levels and A/D ratios increased in patients with MCAD deficiency or GA2 when compared with normal subjects. Reverse-phase HPLC confirmed that n-octanoylated ghrelin levels were elevated in these patients.
  
  
  CONCLUSIONS
  Changing serum medium-chain acylcarnitine levels may affect circulating acyl ghrelin levels, suggesting that acyl-CoA is the substrate for ghrelin acylation.
authors: The First Department of Medicine, Wakayama Medical University, 811-1 Kimi-idera, Wakayama 641-8509, Japan. akamizu@wakayama-med.ac.jp
authors: Takashi
authors: T
authors: Akamizu
authors: Nobuo
authors: N
authors: Sakura
authors: Yosuke
authors: Y
authors: Shigematsu
authors: Go
authors: G
authors: Tajima
authors: Akira
authors: A
authors: Ohtake
authors: Hiroshi
authors: H
authors: Hosoda
authors: Hiroshi
authors: H
authors: Iwakura
authors: Hiroyuki
authors: H
authors: Ariyasu
authors: Kenji
authors: K
authors: Kangawa
mesh_terms: D042964:Acyl-CoA Dehydrogenase / Q000097:blood / Q000172:deficiency / Q000378:metabolism; D000328:Adult; D001774:Blood Chemical Analysis / Q000379:methods; D002331:Carnitine / Q000031:analogs & derivatives / Q000032:analysis / Q000097:blood; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002851:Chromatography, High Pressure Liquid; D005260:Female; D054439:Ghrelin / Q000032:analysis / Q000097:blood* / Q000378:metabolism; D006801:Humans; D008052:Lipid Metabolism, Inborn Errors / Q000097:blood* / Q000378:metabolism; D054069:Multiple Acyl Coenzyme A Dehydrogenase Deficiency / Q000097:blood* / Q000378:metabolism; D011499:Protein Processing, Post-Translational; D055815:Young Adult
chemical_list: D054439:Ghrelin; C116917:acylcarnitine; D042964:Acyl-CoA Dehydrogenase; D002331:Carnitine