Hmdb loader
Survey
Identification
HMDB Protein ID HMDBP08681
Secondary Accession Numbers
  • 14401
Name Prolyl 3-hydroxylase 1
Synonyms
  1. Growth suppressor 1
  2. Leprecan-1
  3. Leucine- and proline-enriched proteoglycan 1
Gene Name LEPRE1
Protein Type Unknown
Biological Properties
General Function Involved in oxidoreductase activity
Specific Function Basement membrane-associated chondroitin sulfate proteoglycan (CSPG). Has prolyl 3-hydroxylase activity catalyzing the post-translational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens, especially types IV and V. May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts.
Pathways Not Available
Reactions
L-proline-[procollagen] + Oxoglutaric acid + Oxygen → trans-3-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2) details
GO Classification
Biological Process
extracellular matrix organization
regulation of ossification
cell growth
collagen fibril organization
negative regulation of cell proliferation
Cellular Component
endoplasmic reticulum lumen
nucleus
proteinaceous extracellular matrix
Function
ion binding
cation binding
metal ion binding
binding
catalytic activity
transition metal ion binding
l-ascorbic acid binding
iron ion binding
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen
vitamin binding
oxidoreductase activity
Molecular Function
oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen
procollagen-proline 3-dioxygenase activity
L-ascorbic acid binding
iron ion binding
Process
metabolic process
oxidation reduction
Cellular Location
  1. Secreted
  2. extracellular space
  3. Endoplasmic reticulum
  4. extracellular matrix
Gene Properties
Chromosome Location 1
Locus 1p34.1
SNPs LEPRE1
Gene Sequence
>2211 bp
ATGGCGGTACGCGCGTTGAAGCTGCTGACCACACTGCTGGCTGTCGTGGCCGCTGCCTCC
CAAGCCGAGGTCGAGTCCGAGGCAGGATGGGGCATGGTGACGCCTGATCTGCTCTTCGCC
GAGGGGACCGCAGCCTACGCGCGCGGGGACTGGCCCGGGGTGGTCCTGAGCATGGAACGG
GCGCTGCGCTCCCGGGCAGCCCTCCGCGCCCTTCGCCTGCGCTGCCGCACCCAGTGTGCC
GCCGACTTCCCGTGGGAGCTGGACCCCGACTGGTCCCCCAGCCCGGCCCAGGCCTCGGGC
GCCGCCGCCCTGCGCGACCTGAGCTTCTTCGGGGGCCTTCTGCGTCGCGCTGCCTGCCTG
CGCCGCTGCCTCGGGCCGCCGGCCGCCCACTCGCTCAGCGAAGAGATGGAGCTGGAGTTC
CGCAAGCGGAGCCCCTACAACTACCTGCAGGTCGCCTACTTCAAGATCAACAAGTTGGAG
AAAGCTGTTGCTGCAGCACACACCTTCTTCGTGGGCAATCCTGAGCACATGGAAATGCAG
CAGAACCTAGACTATTACCAAACCATGTCTGGAGTGAAGGAGGCCGACTTCAAGGATCTT
GAGACTCAACCCCATATGCAAGAATTTCGACTGGGAGTGCGACTCTACTCAGAGGAACAG
CCACAGGAAGCTGTGCCCCACCTAGAGGCGGCGCTGCAAGAATACTTTGTGGCCTATGAG
GAGTGCCGTGCCCTCTGCGAAGGGCCCTATGACTACGATGGCTACAACTACCTTGAGTAC
AACGCTGACCTCTTCCAGGCCATCACAGATCATTACATCCAGGTCCTCAACTGTAAGCAG
AACTGTGTCACGGAGCTTGCTTCCCACCCAAGTCGAGAGAAGCCCTTTGAAGACTTCCTC
CCATCGCATTATAATTATCTGCAGTTTGCCTACTATAACATTGGGAATTATACACAGGCT
GTTGAATGTGCCAAGACCTATCTTCTCTTCTTCCCCAATGACGAGGTGATGAACCAAAAT
TTGGCCTATTATGCAGCTATGCTTGGAGAAGAACACACCAGATCCATCGGCCCCCGTGAG
AGTGCCAAGGAGTACCGACAGCGAAGCCTACTGGAAAAAGAACTGCTTTTCTTCGCTTAT
GATGTTTTTGGAATTCCCTTTGTGGATCCGGATTCATGGACTCCAGAAGAAGTGATTCCC
AAGAGATTGCAAGAGAAACAGAAGTCAGAACGGGAAACAGCCGTACGCATCTCCCAGGAG
ATTGGGAACCTTATGAAGGAAATCGAGACCCTTGTGGAAGAGAAGACCAAGGAGTCACTG
GATGTGAGCAGACTGACCCGGGAAGGTGGCCCCCTGCTGTATGAAGGCATCAGTCTCACC
ATGAACTCCAAACTCCTGAATGGTTCCCAGCGGGTGGTGATGGACGGCGTAATCTCTGAC
CACGAGTGTCAGGAGCTGCAGAGACTGACCAATGTGGCAGCAACCTCAGGAGATGGCTAC
CGGGGTCAGACCTCCCCACATACTCCCAATGAAAAGTTCTATGGTGTCACTGTCTTCAAA
GCCCTCAAGCTGGGGCAAGAAGGCAAAGTTCCTCTGCAGAGTGCCCACCTGTACTACAAC
GTGACGGAGAAGGTGCGGCGCATCATGGAGTCCTACTTCCGCCTGGATACGCCCCTCTAC
TTTTCCTACTCTCATCTGGTGTGCCGCACTGCCATCGAAGAGGTCCAGGCAGAGAGGAAG
GATGATAGTCATCCAGTCCACGTGGACAACTGCATCCTGAATGCCGAGACCCTCGTGTGT
GTCAAAGAGCCCCCAGCCTACACCTTCCGCGACTACAGCGCCATCCTTTACCTAAATGGG
GACTTCGATGGCGGAAACTTTTATTTCACTGAACTGGATGCCAAGACCGTGACGGCAGAG
GTGCAGCCTCAGTGTGGAAGAGCCGTGGGATTCTCTTCAGGCACTGAAAACCCACATGGA
GTGAAGGCTGTCACCAGGGGGCAGCGCTGTGCCATCGCCCTGTGGTTCACCCTGGACCCT
CGACACAGCGAGCGGGACAGGGTGCAGGCAGATGACCTGGTGAAGATGCTCTTCAGCCCA
GAAGAGATGGACCTCTCCCAGGAGCAGCCCCTGGATGCCCAGCAGGGCCCCCCCGAACCT
GCACAAGAGTCTCTCTCAGGCAGTGAATCGAAGCCCAAGGATGAGCTATGA
Protein Properties
Number of Residues 736
Molecular Weight 78921.41
Theoretical pI 5.583
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Prolyl 3-hydroxylase 1
MAVRALKLLTTLLAVVAAASQAEVESEAGWGMVTPDLLFAEGTAAYARGDWPGVVLSMER
ALRSRAALRALRLRCRTQCAADFPWELDPDWSPSPAQASGAAALRDLSFFGGLLRRAACL
RRCLGPPAAHSLSEEMELEFRKRSPYNYLQVAYFKINKLEKAVAAAHTFFVGNPEHMEMQ
QNLDYYQTMSGVKEADFKDLETQPHMQEFRLGVRLYSEEQPQEAVPHLEAALQEYFVAYE
ECRALCEGPYDYDGYNYLEYNADLFQAITDHYIQVLNCKQNCVTELASHPSREKPFEDFL
PSHYNYLQFAYYNIGNYTQAVECAKTYLLFFPNDEVMNQNLAYYAAMLGEEHTRSIGPRE
SAKEYRQRSLLEKELLFFAYDVFGIPFVDPDSWTPEEVIPKRLQEKQKSERETAVRISQE
IGNLMKEIETLVEEKTKESLDVSRLTREGGPLLYEGISLTMNSKLLNGSQRVVMDGVISD
HECQELQRLTNVAATSGDGYRGQTSPHTPNEKFYGVTVFKALKLGQEGKVPLQSAHLYYN
VTEKVRRIMESYFRLDTPLYFSYSHLVCRTAIEEVQAERKDDSHPVHVDNCILNAETLVC
VKEPPAYTFRDYSAILYLNGDFDGGNFYFTELDAKTVTAEVQPQCGRAVGFSSGTENPHG
VKAVTRGQRCAIALWFTLDPRHSERDRVQADDLVKMLFSPEEMDLSQEQPLDAQQGPPEP
AQESLSGSESKPKDEL
GenBank ID Protein 186928835
UniProtKB/Swiss-Prot ID Q32P28
UniProtKB/Swiss-Prot Entry Name P3H1_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_022356.3
GeneCard ID LEPRE1
GenAtlas ID LEPRE1
HGNC ID HGNC:19316
References
General References
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  4. Otsuki T, Ota T, Nishikawa T, Hayashi K, Suzuki Y, Yamamoto J, Wakamatsu A, Kimura K, Sakamoto K, Hatano N, Kawai Y, Ishii S, Saito K, Kojima S, Sugiyama T, Ono T, Okano K, Yoshikawa Y, Aotsuka S, Sasaki N, Hattori A, Okumura K, Nagai K, Sugano S, Isogai T: Signal sequence and keyword trap in silico for selection of full-length human cDNAs encoding secretion or membrane proteins from oligo-capped cDNA libraries. DNA Res. 2005;12(2):117-26. [PubMed:16303743 ]
  5. Kaul SC, Sugihara T, Yoshida A, Nomura H, Wadhwa R: Gros1, a potential growth suppressor on chromosome 1: its identity to basement membrane-associated proteoglycan, leprecan. Oncogene. 2000 Jul 27;19(32):3576-83. [PubMed:10951563 ]
  6. Cabral WA, Chang W, Barnes AM, Weis M, Scott MA, Leikin S, Makareeva E, Kuznetsova NV, Rosenbaum KN, Tifft CJ, Bulas DI, Kozma C, Smith PA, Eyre DR, Marini JC: Prolyl 3-hydroxylase 1 deficiency causes a recessive metabolic bone disorder resembling lethal/severe osteogenesis imperfecta. Nat Genet. 2007 Mar;39(3):359-65. Epub 2007 Feb 4. [PubMed:17277775 ]