   1.14.11.4 | more |
the endoplasmic reticulum complex including SC65 and prolyl 3-hydroxylase 3affects the activity of lysyl-hydroxylase 1 potentially through interactions with the enzyme and/or cyclophilin B. Loss of Sc65 in the mouse results in instability of this complex, altered collagen lysine hydroxylation and cross-linking leading to connective tissue defects that include low bone mass and skin fragility, while it has no effect on prolyl 3-hydroxylation. CRTAP, a non-enzymic member of the Leprecan family of proteins which includes Synaptonemal Complex 65 (SC65) and the prolyl 3-hydroxylases (P3H1, P3H2 and P3H3), is an essential third subunit of a complex with prolyl 3-hydroxylase 1 (aka LEPRECAN) and cyclophilin B (CYPB) in the endoplasmic reticulum, forming the so-called collagen prolyl 3-hydroxylation complex |
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