GGCX gamma-carboxylates BGLAP(24-100) (pro-osteocalcin)

Stable Identifier
Reaction [transition]
Homo sapiens
pro-osteocalciin, uncarboxylated + 3 CO2 + 3 O2 + 3 vitamin K hydroquinone -> gamma-carboxylated pro-osteocalcin + 3 H2O + 3 vitamin K epoxide
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GGCX (gamma glutamyl carboxylase) in the endoplasmic reticulum gamma-carboxylates three glutamate residues on BGLAP(24-100) (pro-osteocalcin). MK4 (vitamin K hydroquinone) is oxidized to MK4 epoxide in the process (Berkner 2000; Ferron et al. 2015; Furie et al. 1999; Hauschka et al. 1989; Morris et al. 1995; oser et al. 1980; Stenina et al. 2001).

Literature References
PubMed ID Title Journal Year
10068650 Vitamin K-dependent biosynthesis of gamma-carboxyglutamic acid

Furie, B, Bouchard, BA, Furie, BC

Blood 1999
10917896 The vitamin K-dependent carboxylase

Berkner, KL

J Nutr 2000
6967872 Isolation and sequence of the vitamin K-dependent protein from human bone. Undercarboxylation of the first glutamic acid residue

Poser, JW, Ling, NC, Price, PA, Esch, FS

J. Biol. Chem. 1980
2664828 Osteocalcin and matrix Gla protein: vitamin K-dependent proteins in bone

Lian, JB, Cole, DE, Gundberg, CM, Hauschka, PV

Physiol. Rev. 1989
8530480 Processive post-translational modification. Vitamin K-dependent carboxylation of a peptide substrate.

Stafford, DW, Wright, DJ, Stevens, RD, Morris, DP

J Biol Chem 1995
25753038 GGCX and VKORC1 inhibit osteocalcin endocrine functions

Oury, F, Germain, A, Lacombe, J, Ferron, M, Karsenty, G

J. Cell Biol. 2015
11513608 Tethered processivity of the vitamin K-dependent carboxylase: factor IX is efficiently modified in a mechanism which distinguishes Gla's from Glu's and which accounts for comprehensive carboxylation in vivo

Stenina, O, Pudota, BN, Berkner, KL, McNally, BA, Hommema, EL

Biochemistry 2001
Catalyst Activity

gamma-glutamyl carboxylase activity of GGCX [endoplasmic reticulum membrane]

Orthologous Events
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