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  • deficient
  • The incidence of 21-hydroxylase deficient CAH detectable in childhood is about 1 in 15,000 births. (wikipedia.org)
  • When it occurs it resembles the salt-wasting of severe 21-hydroxylase deficient CAH: poor weight gain and vomiting in the first weeks of life progress and culminate in life-threatening dehydration, hyponatremia, hyperkalemia, and metabolic acidosis in the first month. (wikipedia.org)
  • These androgens produce effects that are similar to those of 21-hydroxylase deficient CAH. (wikipedia.org)
  • Although "nonclassic" forms causing hirsutism and menstrual irregularities and appropriate steroid elevations have been reported, most have not had verifiable mutations and mild 11β-hydroxylase deficient CAH is currently considered a very rare cause of hirsutism and infertility. (wikipedia.org)
  • 11β-OH CAH resembles 21-hydroxylase deficient CAH in its androgenic manifestations: partial virilization and ambiguous genitalia of genetically female infants, childhood virilization of both sexes, and rarer cases of virilization or infertility of adolescent and adult women. (wikipedia.org)
  • enzymology
  • In enzymology, a deoxysarpagine hydroxylase (EC 1.14.13.91) is an enzyme that catalyzes the chemical reaction 10-deoxysarpagine + NADPH + H+ + O2 ⇌ {\displaystyle \rightleftharpoons } sarpagine + NADP+ + H2O The 4 substrates of this enzyme are 10-deoxysarpagine, NADPH, H+, and O2, whereas its 3 products are sarpagine, NADP+, and H2O. (wikipedia.org)
  • In enzymology, an ethylbenzene hydroxylase (EC 1.17.99.2) is an enzyme that catalyzes the chemical reaction ethylbenzene + H2O + acceptor ⇌ {\displaystyle \rightleftharpoons } (S)-1-phenylethanol + reduced acceptor The 3 substrates of this enzyme are ethylbenzene, H2O, and acceptor, whereas its two products are (S)-1-phenylethanol and reduced acceptor. (wikipedia.org)
  • In enzymology, a proline 3-hydroxylase (EC 1.14.11.28) is an enzyme that catalyzes the chemical reaction L-proline + 2-oxoglutarate + O2 ⇌ {\displaystyle \rightleftharpoons } cis-3-hydroxy-L-proline + succinate + CO2 The 3 substrates of this enzyme are L-proline, 2-oxoglutarate, and O2, whereas its 3 products are cis-3-hydroxy-L-proline, succinate, and CO2. (wikipedia.org)
  • PMID
  • Junctate is an integral ER/SR membrane calcium binding protein, which comes from an alternatively spliced form of the same gene that generates aspartyl beta-hydroxylase and junctin [ PMID: 11735129 ]. (ebi.ac.uk)
  • Protein
  • This domain is found in members of the junctin, junctate and aspartyl beta-hydroxylase protein families. (ebi.ac.uk)
  • This enzyme catalyses the following chemical reaction abieta-7,13-diene + NADPH + H+ + O2 ⇌ {\displaystyle \rightleftharpoons } abieta-7,13-dien-18-ol + NADP+ + H2O Abietadiene hydroxylase is a heme-thiolate protein (P-450). (wikipedia.org)
  • The enzymatic structure of the human cholesterol-24 hydroxylase was determined via crystallography at the Stanford Synchrotron Radiation Lightsource, and was shown to be a 57kDa (500 residues) monomeric heme-containing protein bound to the endoplasmic reticulum in neurons. (wikipedia.org)
  • converts
  • Specifically, 21-hydroxylase converts progesterone and 17α-hydroxyprogesterone into 11-deoxycorticosterone and 11-deoxycortisol, respectively, by hydroxylating at the C21 position. (wikipedia.org)
  • bound
  • The x-ray crystal structure for human 21-hydroxylase, with bound progesterone, was realized and published in 2015, providing opportunity for further study. (wikipedia.org)