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LEPRE1 Rabbit pAb

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
IHC-P, IHC-F, IF, E
Primary Accession Q32P28
Other Accession Q32P28
Host Rabbit
Clonality Polyclonal
Calculated MW 83394 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human LEPRE1
Epitope Specificity 351-450/736
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY Belongs to the leprecan family. Contains 1 Fe2OG dioxygenase domain. Contains 4 TPR repeats.
Post-translational modifications O-glycosylated; chondroitin sulfate.
DISEASE Defects in LEPRE1 are the cause of osteogenesis imperfecta type 8 (OI8) [MIM:610915]. 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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions 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]
Additional Information
Gene ID 64175
Other Names GROS1; LEPRE1; OI8; 2410024C15Rik; P3H1_HUMAN; P3H1; Growth suppressor 1; Leucine- and proline-enriched proteoglycan 1 (Leprecan-1); 1.14.11.7; P3H1_MOUSE; P3H1_RAT; prolyl 3-hydroxylase 1; leucine proline-enriched proteoglycan (leprecan) 1; procollagen-proline 3-dioxygenase
Dilution IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name P3H1 (HGNC:19316)
Function Has prolyl 3-hydroxylase activity and catalyzes the post- translational formation of 3-hydroxyproline in -Xaa-Pro-Gly- sequences in pro-collagen chains, a critical step for the formation of mature collagen trimers (PubMed:39245686). May be involved in the secretory pathway of cells. Has growth suppressive activity in fibroblasts.
Cellular Location [Isoform 1]: Endoplasmic reticulum
Research Areas
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