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Prolyl 4-Hydrolase, alpha OKDB#: 918
 Symbols: Prolyl 4-Hydrolase, alpha Species: human
 Synonyms: PROLYL 4-HYDROXYLASE, ALPHA-1 SUBUNIT| PROLYL 4-HYDROXYLASE, ALPHA SUBUNIT, P4HA|  Locus: 10q21.3-q23.1 in Homo sapiens


For retrieval of Nucleotide and Amino Acid sequences please go to: OMIM Entrez Gene
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General Comment Prolyl 4-hydroxylase (EC 1.14.11.2 ) plays a central role in collagen synthesis. It catalyzes the formation of 4-hydroxyproline in collagens by hydroxylation of proline residues in peptide linkages. The 4-hydroxyproline residues are essential for the folding of the newly synthesized procollagen polypeptide chain into triple helical molecules. The active enzyme is a tetramer of 2 alpha and 2 beta subunits with a molecular weight of about 240,000. The beta subunit (P4HB; ) is identical to the enzyme disulfide isomerase (EC 5.3.4.1 ) and a major cellular thyroid-binding protein. The alpha subunit probably contributes a major part of the catalytic site of the enzyme. Helaakoski et al. (1989) isolated cDNA clones for the alpha subunit. They found that the clones encode a polypeptide of 517 amino acid residues and a signal peptide of 17 amino acids.

General function Enzyme, Oxidoreductase
Comment
Cellular localization Extracellular Matrix, Secreted
Comment
Ovarian function Ovulation
Comment Nagai R et al 1998 reported spatio-temporal changes of prolyl 4-hydroxylase in granulosa cells during ovulation in eCG-hCG-treated immature rat ovaries. The immunolocalization of prolyl 4-hydroxylase (PHase), a key enzyme of collagen synthesis, and the effects of anti-progesterone RU486 on PHase during the ovulatory process in eCG-hCG-treated immature rat ovaries were studied to investigate the mechanisms of tissue repair in follicle walls after follicular rupture. No obvious immunolocalization of PHase was observed in theca cells throughout the ovulatory process except just after follicular rupture. In contrast, in granulosa cells, PHase was first observed at 9 h after the hCG injection, and the staining intensity apparently increased from 9 to 15 h, especially around the apex of preovulatory follicles and the orifice of ruptured follicles. Consistent with these observations, PHase concentration in granulosa cells isolated from the ovaries significantly increased by 9 h (0.45 +/- 0.03 pg per cell), and reached a peak at 15 h (0.66 +/- 0.06 pg per cell) after the hCG injection. This peak was inhibited when 20 mg RU486 kg-1 was administered at 8 h. The results suggest that PHase expressed in granulosa cells may play an important role in the repair of ruptured follicle walls, via progesterone-dependent PHase production.
Expression regulated by LH, Steroids
Comment
Ovarian localization Granulosa, Theca, Surface epithelium
Comment Auersperg N, reported that prolyl 4-hydroxylase is present in cultured human ovarian surface epithelial cells.
Follicle stages Preovulatory
Comment
Phenotypes
Mutations 0 mutations
Genomic Region show genomic region
Phenotypes and GWAS show phenotypes and GWAS
Links
OMIM (Online Mendelian Inheritance in Man: an excellent source of general gene description and genetic information.)
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created: April 16, 2000, midnight by: hsueh   email:
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last update: Aug. 14, 2001, 10:30 a.m. by: hsueh    email:



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