LEPRE1 blocking peptide

LEPRE1 Blocking Peptide (Center)

• Gene Names: P3H1; OI8; GROS1; LEPRE1
• Synonyms: LEPRE1; LEPRE1 Blocking Peptide (Center); Prolyl 3-hydroxylase 1; Growth suppressor 1; Leucine- and proline-enriched proteoglycan 1; Leprecan-1; GROS1; P3H1; LEPRE1 blocking peptide

• Specificity: The synthetic peptide sequence is selected from aa 499-513 of HUMAN LEPRE1
• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 736

• Cellular Location: Isoform 1: Endoplasmic reticulum.
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for LEPRE1 blocking peptide

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.

NCBI and Uniprot Product Information

• NCBI GI #: 109892809
• NCBI GeneID: 64175
• UniProt Accession #: Q32P28; Q7KZR4; Q96BR8; Q96SK8; Q96SL5; Q96SN3; Q9H6K3; Q9HC86; Q9HC87
• Molecular Weight: 78,922 Da
• NCBI Official Full Name: Prolyl 3-hydroxylase 1
• NCBI Official Synonym Full Names: prolyl 3-hydroxylase 1
• NCBI Official Symbol: P3H1
• NCBI Official Synonym Symbols: OI8; GROS1; LEPRE1
• NCBI Protein Information: prolyl 3-hydroxylase 1
• UniProt Protein Name: Prolyl 3-hydroxylase 1
• UniProt Gene Name: P3H1
• UniProt Synonym Gene Names: Leprecan-1
• UniProt Entry Name: P3H1_HUMAN

NCBI Description

This gene encodes an enzyme that is a member of the collagen prolyl hydroxylase family. These enzymes are localized to the endoplasmic reticulum and their activity is required for proper collagen synthesis and assembly. Mutations in this gene are associated with osteogenesis imperfecta type VIII. Three alternatively spliced transcript variants encoding different isoforms have been described. Other variants may exist, but their biological validity has not been determined. [provided by RefSeq, Aug 2011]

Uniprot Description

LEPRE1: 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. Defects in LEPRE1 are the cause of osteogenesis imperfecta type 8 (OI8). A connective tissue disorder characterized by disproportionate short stature, severe osteoporosis, shortening of the long bones, white sclerae, a round face and a short barrel-shaped chest. Belongs to the leprecan family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 1.14.11.7; Secreted, signal peptide; Oxidoreductase; Cell development/differentiation; Secreted

Chromosomal Location of Human Ortholog: 1p34.1

Cellular Component: endoplasmic reticulum; endoplasmic reticulum lumen; membrane

Molecular Function: collagen binding; procollagen-proline 3-dioxygenase activity; protein complex binding

Biological Process: collagen metabolic process; protein amino acid hydroxylation; protein folding; protein stabilization; regulation of protein secretion

Disease: Osteogenesis Imperfecta, Type Viii