Английская Википедия:25-Hydroxyvitamin D 1-alpha-hydroxylase

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Шаблон:Short description

Шаблон:Infobox gene Шаблон:Infobox enzyme

25-Hydroxyvitamin D 1-alpha-hydroxylase (VD 1A hydroxylase) also known as calcidiol 1-monooxygenase [1] or cytochrome p450 27B1 (CYP27B1) or simply 1-alpha-hydroxylase is a cytochrome P450 enzyme that in humans is encoded by the CYP27B1 gene.[2][3][4]

VD 1A hydroxylase is located in the proximal tubule of the kidney and a variety of other tissues, including skin (keratinocytes), immune cells,[5] and bone (osteoblasts).[6]

Reactions

The enzyme catalyzes the hydroxylation of calcifediol to calcitriol (the bioactive form of Vitamin D):[7]

calcidiol + 2 reduced adrenodoxin + 2 H+ + O2 Шаблон:Eqm calcitriol + 2 oxidized adrenodoxin + H2O

The enzyme is also able to oxidize ercalcidiol (25-OH D2) to ercalcitriol, secalciferol to calcitetrol, and 25-hydroxy-24-oxocalciol to (1S)-1,25-dihydroxy-24-oxocalciol.[8]

Clinical significance

Loss-of-function mutations in CYP27B1 cause Vitamin D-dependent rickets, type IA.[9]

Interactive pathway map

Шаблон:VitaminDSynthesis WP1531

References

Шаблон:Reflist

Further reading

Шаблон:Refbegin

Шаблон:Refend

External links

Шаблон:Cytochrome P450 Шаблон:Metabolism of vitamins, coenzymes, and cofactors Шаблон:Dioxygenases Шаблон:Enzymes Шаблон:Portal bar

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  1. ↑ Шаблон:Cite web
  2. ↑ Шаблон:Cite web
  3. ↑ Шаблон:Cite journal
  4. ↑ Шаблон:Cite journal
  5. ↑ Шаблон:Cite journalШаблон:Dead link
  6. ↑ Шаблон:Cite journal
  7. ↑ Шаблон:Cite journal
  8. ↑ Шаблон:Cite journal
  9. ↑ Шаблон:Cite web
Источник — http://wikihandbk.com/ruwiki/index.php?title=Английская_Википедия:25-Hydroxyvitamin_D_1-alpha-hydroxylase&oldid=12840608