HSG095

• TYPE: 2D
• ACCESSION: HSG095
• TITLE: Succinyl-CoA:3-ketoacid-coenzyme A transferase
  • EC 2.8.3.5
  • Succinyl CoA:3-oxoacid CoA-transferase
• LOCALIZATION: mitochondrial
• GENERAL-DATA: 00004
• IDENTIFICATION: MALDI-TOF MS
• SPOT-DATA
  • pI: 4.3
  • MW: 51.1 kDa
  • Sequence-exp.:
    • N-terminal
    • MS-Fit: HSG095
  • Entry name: SCOT_HUMAN
  • Gene-name: OXCT OR SCOT
  • pI-calc.: 6.00
  • MW-calc.: 52089.89
  • Number-of-residues: 481
• COMMENTS:

  FUNCTION: KEY ENZYME FOR KETONE BODY CATABOLISM. TRANSFERS THE COA MOIETY FROM SUCCINATE TO ACETOACETATE. FORMATION OF THE ENZYME-COA INTERMEDIATE PROCEEDS VIA AN UNSTABLE ANHYDRIDE SPECIES FORMED BETWEEN THE CARBOXYLATE GROUPS OF THE ENZYME AND SUBSTRATE. * CATALYTIC ACTIVITY: Succinyl-CoA + a 3-oxo acid = succinate + a 3-oxo-acyl-CoA. * PATHWAY: FIRST STEP OF KETOLYSIS IN EXTRAHEPATIC TISSUES. * SUBUNIT: Homodimer. * SUBCELLULAR LOCATION: Mitochondrial matrix. * TISSUE SPECIFICITY: ABUNDANT IN HEART, FOLLOWED IN ORDER BY KIDNEY, BRAIN, AND MUSCLE, WHEREAS IN LIVER IT IS UNDETECTABLE; ALSO DETECTABLE IN LEUKOCYTES AND FIBROBLASTS. * DISEASE: DEFECTS IN OXCT ARE THE CAUSE OF A FORM OF HYPERKETONEMIA WITH EPISODIC SEVERE KETOACIDOSIS. * SIMILARITY: BELONGS TO THE 3-OXOACID COA-TRANSFERASE FAMILY.

• REFERENCES
  • PIR:
  • Swiss-Prot: P55809
    • NiceProt View of SWISS-PROT: P55809
  • NCBI:
  • MEDLINE:

23-Jan-03

Last Update 13-Mar-03

Spot Data Index

List of HSG proteins by accesion number

List of HSG proteins by

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