anti-Leucine Proline-Enriched Proteoglycan (Leprecan) 1 (LEPRE1) Anticorps

LEPRE1 encodes an enzyme that is a member of the collagen prolyl hydroxylase family. De plus, nous expédions LEPRE1 Kits (7) et LEPRE1 Protéines (6) et beaucoup plus de produits pour cette protéine.

afficher tous les anticorps Gène GeneID UniProt
LEPRE1 64175 Q32P28
Anti-Rat LEPRE1 LEPRE1 114200 Q9R1J8
LEPRE1 56401 Q3V1T4
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Top anti-LEPRE1 Anticorps sur anticorps-enligne.fr

Showing 10 out of 46 products:

Catalogue No. Reactivité Hôte Conjugué Application Images Quantité Livraison Prix Détails
Humain Souris Inconjugué WB LEPRE1 MaxPab polyclonal antibody. Western Blot analysis of LEPRE1 expression in human placenta. Western Blot analysis of LEPRE1 expression in transfected 293T cell line by LEPRE1 MaxPab polyclonal antibody.Lane 1: LEPRE1 transfected lysate(42.9 KDa).Lane 2: Non-transfected lysate. 50 μg 11 to 12 Days
$440.00
Détails
Humain Souris Inconjugué IF, IHC (p), ELISA, WB Immunoperoxidase of monoclonal antibody to LEPRE1 on formalin-fixed paraffin-embedded human esophagus. [antibody concentration 6 ug/ml] Immunofluorescence of monoclonal antibody to LEPRE1 on HeLa cell. [antibody concentration 10 ug/ml] 100 μg 11 to 12 Days
$440.00
Détails
Humain Souris Inconjugué ELISA, IF, IHC, IHC (p), WB 100 μg 11 to 14 Days
$551.83
Détails
Humain Souris Inconjugué FACS, WB HEK293T cells transfected with either RC222535 overexpress plasmid (Red) or empty vector control plasmid (Blue) were immunostained by anti-LEPRE1 antibody (ABIN2455936), and then analyzed by flow cytometry. HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY LEPRE1 (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 µg per lane) were separated by SDS-PAGE and immunoblotted with anti-LEPRE1. 0.1 mL 2 to 3 Days
$462.00
Détails
Humain Souris Inconjugué FACS, IF, WB HEK293T cells transfected with either RC222535 overexpress plasmid (Red) or empty vector control plasmid (Blue) were immunostained by anti-LEPRE1 antibody (ABIN2455943), and then analyzed by flow cytometry. Anti-LEPRE1 mouse monoclonal antibody (ABIN2455943) immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY LEPRE1 (RC222535). 0.1 mL 2 to 3 Days
$462.00
Détails
Humain Souris Inconjugué FACS, IHC, IHC (p), WB 100 μL 11 to 14 Days
$522.50
Détails
Humain Lapin Inconjugué WB Western blot analysis of extract of various cells, using LEPRE1 antibody. Western blot analysis of extracts of various cell lines, using LEPRE1 antibody (ABIN4904219) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 30s. 100 μL 11 to 13 Days
$366.77
Détails
Humain Lapin Inconjugué WB   100 μL 2 to 3 Days
$289.00
Détails
Humain Lapin Alkaline Phosphatase (AP) IHC, ELISA   200 μL 11 to 14 Days
$1,013.83
Détails
Humain Lapin APC IHC, ELISA   200 μL 11 to 14 Days
$1,013.83
Détails

anti-LEPRE1 Anticorps mieux référencés

  1. Human Polyclonal LEPRE1 Primary Antibody pour WB - ABIN528557 : Willaert, Malfait, Symoens, Gevaert, Kayserili, Megarbane, Mortier, Leroy, Coucke, De Paepe: Recessive osteogenesis imperfecta caused by LEPRE1 mutations: clinical documentation and identification of the splice form responsible for prolyl 3-hydroxylation. dans Journal of medical genetics 2009 (PubMed)
    Show all 9 Pubmed References

  2. Human Monoclonal LEPRE1 Primary Antibody pour IF, IHC (p) - ABIN566242 : Cabral, Chang, Barnes, Weis, Scott, Leikin, Makareeva, Kuznetsova, Rosenbaum, Tifft, Bulas, Kozma, Smith, Eyre, Marini: Prolyl 3-hydroxylase 1 deficiency causes a recessive metabolic bone disorder resembling lethal/severe osteogenesis imperfecta. dans Nature genetics 2007 (PubMed)
    Show all 4 Pubmed References

Plus d’anticorps contre LEPRE1 partenaires d’interaction

Human Leucine Proline-Enriched Proteoglycan (Leprecan) 1 (LEPRE1) interaction partners

  1. P3H1 mutation is associated with Non-Lethal Type VIII Osteogenesis Imperfecta.

  2. This study enhances our knowledge about the mutational pattern of the LEPRE1, CRTAP, and PPIB genes. LEPRE1 should be the first gene analyzed in mutation detection studies in patients with recessive OI.

  3. An additional function of the rough endoplasmic reticulum protein complex prolyl 3-hydroxylase 1.cartilage-associated protein.cyclophilin B: the CXXXC motif reveals disulfide isomerase activity in vitro.

  4. This is the first report of a mutation in LEPRE1 that eliminates only the KDEL ER-retrieval sequence, whereas other functional domains remain intact

  5. 0.4% of Mid-Atlantic African Americans and 1.48% West Africans carry mutation in LEPRE1 which causes lethal recessive osteogenesis imperfecta.

  6. Mutation analyses were performed for COL1A1, COL1A2, CRTAP, and LEPRE1 in a cohort of 58 unrelated Chinese patients with osteogenesis imperfecta.

  7. We report a large consanguineous Turkish family in which multiple individuals are affected with autosomal recessive lethal or severe osteogenesis imperfecta (OI) due to a novel homozygous LEPRE1 mutation

  8. Null mutations in LEPRE1 and CRTAP cause severe recessive osteogenesis imperfecta.

  9. CRTAP and P3H1 are mutually stabilized in the collagen prolyl 3-hydroxylation complex in endoplasmic reticulum.

  10. Screening of 78 subjects diagnosed with osteogenesis imperfecta type II or III, identified three probands with mutations in CRTAP and 16 with mutations in LEPRE1.

  11. Mutations in CRTAP and LEPRE1 are found in 3 patients with type II osteogenesis imperfecta.

  12. Findings suggest that the 3-hydroxylation function of P3H1 is restricted to the 736AA splice form.

Mouse (Murine) Leucine Proline-Enriched Proteoglycan (Leprecan) 1 (LEPRE1) interaction partners

  1. P3H1 deficiency leads to decreased deposition of extracellular matrix by osteoblasts and increased incorporation of mineral into the matrix.

  2. we generated knock-in mice carrying a single amino acid substitution in the catalytic site of P3h1 (Lepre1(H662A) ). This substitution abolished P3h1 activity but retained ability to form a complex with Crtap and thus the collagen chaperone

  3. An additional function of the rough endoplasmic reticulum protein complex prolyl 3-hydroxylase 1.cartilage-associated protein.cyclophilin B: the CXXXC motif reveals disulfide isomerase activity in vitro.

  4. The absence of 3-hydroxyproline and/or the increased glycosylation of hydroxylysine in type I collagen disturbs the lateral growth of the fibrils.

  5. The LEPRE1 knockout mouse is a valuable model system to investigate the mechanism of hearing loss in recessive osteogenesis imperfecta.

  6. Prolyl 3-hydroxylase 1 null mice display abnormalities in fibrillar collagen-rich tissues such as tendons, skin, and bones

  7. Study found that all 3 mouse Leprecan genes Lepre1, leprel1 and leprel2 are expressed in the notochord; additionally, they are expressed in dorsal root ganglia, limb buds, branchial arches, and developing kidneys.

  8. This report characterizes expression of the P3H1, P3H2 and P3H3 genes in embryonic and adult mice.

LEPRE1 profil antigène

Profil protéine

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.

Gene names and symbols associated with LEPRE1

  • prolyl 3-hydroxylase 1 (P3H1) anticorps
  • prolyl 3-hydroxylase 1 (P3h1) anticorps
  • prolyl 3-hydroxylase 1 L homeolog (p3h1.L) anticorps
  • prolyl 3-hydroxylase 1 (p3h1) anticorps
  • 2410024C15Rik anticorps
  • Gros1 anticorps
  • LEPRE1 anticorps
  • MGC84556 anticorps
  • OI8 anticorps
  • P3H1 anticorps
  • sb:cb953 anticorps

Protein level used designations for LEPRE1

growth suppressor 1 , leprecan , leucine- and proline-enriched proteoglycan 1 , prolyl 3-hydroxylase 1 , growth supressor 1 , leprecan 1 , leprecan-1 , leprecan-1 homolog , leucine- and proline-enriched proteoglycan 1 homolog , leucine proline-enriched proteoglycan (leprecan) 1 , prolyl 3-hydroxylase 1-like

GENE ID SPECIES
64175 Homo sapiens
114200 Rattus norvegicus
414142 Gallus gallus
414447 Xenopus laevis
456811 Pan troglodytes
539976 Bos taurus
553321 Danio rerio
606980 Canis lupus familiaris
697917 Macaca mulatta
100036679 Xenopus (Silurana) tropicalis
100402370 Callithrix jacchus
100454619 Pongo abelii
100620503 Sus scrofa
56401 Mus musculus
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