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Hexokinase-1 (hexokinase type I, HK I, brain form hexokinase, HK1)
Function:
- carbohydrate metabolism, hexose metabolism
- allosteric enzyme inhibited by its product Glc-6-P
ATP + D-hexose ADP + D-hexose 6-phosphate
Structure:
- monomer
- N- & C-terminal halves show extensive sequence similarity to each other
- catalytic activity is associated with the C-terminus
- regulatory function is associated with the N-terminus hydrophobic N-terminal sequence may be involved in membrane binding
- belongs to the hexokinase family
Compartment:
- mitochondrial outer membrane
Alternative splicing: named isoforms=4
Expression:
- isoform 2 is erythrocyte specific
- isoform 3 & isoform 4 are testis-specific
Pathology:
- defects in HK1 are the cause of hexokinase deficiency
Notes:
- in vertebrates there are 4 major glucose-phosphorylating isoenzymes, designated hexokinase 1, 2, 3 & 4 (glucokinase)
General
hexokinase
mitochondrial membrane protein
Properties
SIZE: entity length = 917 aa
MW = 102 kD
COMPARTMENT: mitochondria
MOTIF: Hydrophobic {1-12}
Regulatory {13-475}
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 84-89
binding site
SITE: 149-175
FOR-BINDING-OF: glucose
active site
SITE: 476-917
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 532-537
ATP-binding site
NAME: ATP-binding site
SITE: 558-558
binding site
SITE: 597-623
FOR-BINDING-OF: glucose
Database Correlations
OMIM correlations
UniProt P19367
PFAM correlations
KEGG correlations
ENZYME 2.7.1.1
References
- UniProt :accession P19367
- Wikipedia; note=Hexokinase entry
http://en.wikipedia.org/wiki/Hexokinase