LEPRE1 Antibody - Azide and BSA Free Summary
Immunogen LEPRE1 (AAH15309, 1 a.a. - 390 a.a.) full-length human protein. MLGEEHTRSIGPRESAKEYRQRSLLEKELLFFAYDVFGIPFVDPDSWTPEEVIPKRLQEKQKSERETAVRISQEIGNLMKEIETLVEEKTKESLDVSRLTREGGPLLYEGISLTMNSKLLNGSQRVVMDGVISDHECQELQRLTNVAATSGDGYRGQTSPHTPNEKFYGVTVFKALKLGQEGKVPLQSAHLYYNVTEKVRRIMESYFRLDTPLYFSYSHLVCRTAIEEVQAERKDDSHPVHVDNCILNAETLVCVKEPPAYTFRDYSAILYLNGDFDGGNFYFTELDAKTVTAEVQPQCGRAVGFSSGTENPHGVKAVTRGQRCAIALWFTLDPRHSERDRVQADDLVKMLFSPEEMDLSQEQPLDAQQGPPEPAQESLSGSESKPKDEL
Specificity LEPRE1 - leucine proline-enriched proteoglycan (leprecan) 1,
Isotype IgG
Clonality Polyclonal
Host Mouse
Gene P3H1
Purity IgG purified
Innovator's Reward Test in a species/application not listed above to receive a full credit towards a future purchase.
Applications/Dilutions
Dilutions
Immunocytochemistry/ Immunofluorescence Immunohistochemistry Western Blot 1:500
Application Notes Antibody reactive against transfected lysate and tissue lysate for Western Blot.
Publications
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Packaging, Storage & Formulations
Storage Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
Buffer PBS (pH 7.4)
Preservative No Preservative
Purity IgG purified
Notes
This product is produced by and distributed for Abnova, a company based in Taiwan.
Alternate Names for LEPRE1 Antibody - Azide and BSA Free
Background
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. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq]
Limitations
This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are
guaranteed for 1 year from date of receipt.
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Publications for LEPRE1 Antibody (H00064175-B01P)(11)
We have publications tested in 1 confirmed species: Human.
We have publications tested in 1 application: ICC/IF.
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10 of 11.
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Publications using H00064175-B01P
Applications
Species
Besio R, Garibaldi N, Leoni L et al. Cellular stress due to impairment of collagen prolyl hydroxylation complex is rescued by the chaperone 4-phenylbutyrate Dis Model Mech. 2019-06-20 [PMID: 31171565]
Khan M, Zukowska J, Jung J et al. Dextromethorphan inhibits collagen transport in the endoplasmic reticulum eliciting an anti-fibrotic response inex-vivoandin vitromodels of pulmonary fibrosis bioRxiv 2023-04-20 (ICC/IF, Human)
ICC/IF
Human
Huang Y, Mei L, Lv W et al. Targeted exome sequencing identifies novel compound heterozygous mutations in LEPRE1 in a fetus with osteogenesis imperfecta type VIII. Clin Chim Acta 2016-11-15 [PMID: 27864101]
Cabral WA, Perdivara I, Weis M et al. Abnormal type I collagen post-translational modification and crosslinking in a cyclophilin B KO mouse model of recessive osteogenesis imperfecta. PLoS Genet. 2014-06-26 [PMID: 24968150]
Takagi M, Ishii T, Barnes AM et al. A Novel Mutation in LEPRE1 That Eliminates Only the KDEL ER- Retrieval Sequence Causes Non-Lethal Osteogenesis Imperfecta. PLoS One. 2012-05-15 [PMID: 22615817]
Amor IM, Rauch F, Gruenwald K et al. Severe osteogenesis imperfecta caused by a small in-frame deletion in CRTAP. Am J Med Genet A. 2011-09-30 [PMID: 21964860]
Pyott SM, Schwarze U, Christiansen HE et al. Mutations in PPIB (cyclophilin B) delay type I procollagen chain association and result in perinatal lethal to moderate osteogenesis imperfecta phenotypes. Hum Mol Genet. 2011-02-13 [PMID: 21282188]
Baldridge D, Lennington J, Weis M et al. Generalized connective tissue disease in Crtap-/- mouse. PLoS One 5(5). 2010-05-11 [PMID: 20485499]
Chang W, Barnes AM, Cabral WA et al. Prolyl 3-hydroxylase 1 and CRTAP are mutually stabilizing in the endoplasmic reticulum collagen prolyl 3-hydroxylation complex. Hum Mol Genet;19(2):223-34. 2010-01-15 [PMID: 19846465]
van Dijk FS, Nesbitt IM, Zwikstra EH et al. PPIB mutations cause severe osteogenesis imperfecta. Am J Hum Genet 2009-10-01 [PMID: 19781681]
Willaert A, Malfait F, Symoens S et al. Recessive Osteogenesis Imperfecta caused by LEPRE1 mutations: clinical documentation identification of the splice form responsible for prolyl 3-hydroxylation. J Med Genet 46(4):233-41. 2009-04-01 [PMID: 19088120]
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![Western Blot: LEPRE1 Antibody [H00064175-B01P] - Analysis of LEPRE1 expression in human placenta.](http://images.novusbio.com/fullsize/LEPRE1-Antibody-Western-Blot-H00064175-B01P-img0003.jpg "Western Blot: LEPRE1 Antibody [H00064175-B01P] - Analysis of LEPRE1 expression in human placenta.")
![Western Blot: LEPRE1 Antibody [H00064175-B01P] - Analysis of LEPRE1 expression in transfected 293T cell line by LEPRE1 polyclonal antibody. Lane 1: LEPRE1 transfected lysate(42.9 KDa). Lane 2: Non-transfected lysate.](http://images.novusbio.com/fullsize/LEPRE1-Antibody-Western-Blot-H00064175-B01P-img0004.jpg "Western Blot: LEPRE1 Antibody [H00064175-B01P] - Analysis of LEPRE1 expression in transfected 293T cell line by LEPRE1 polyclonal antibody. Lane 1: LEPRE1 transfected lysate(42.9 KDa). Lane 2: Non-transfected lysate.")
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