Leucine Proline-Enriched Proteoglycan (Leprecan) 1 (LEPRE1) ELISA Kits

LEPRE1 encodes an enzyme that is a member of the collagen prolyl hydroxylase family. Zusätzlich bieten wir Ihnen LEPRE1 Antikörper (47) und LEPRE1 Proteine (6) und viele weitere Produktgruppen zu diesem Protein an.

list all ELISA KIts Gen GeneID UniProt
LEPRE1 56401 Q3V1T4
LEPRE1 64175 Q32P28
LEPRE1 114200 Q9R1J8
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Top LEPRE1 ELISA Kits auf antikoerper-online.de

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Katalog Nr. Reaktivität Sensitivität Bereich Menge Anbieter Lieferzeit Preis Details
Human
96 Tests Anmelden zum Anzeigen 13 bis 17 Tage
788,70 €
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Maus
96 Tests Anmelden zum Anzeigen 13 bis 17 Tage
788,70 €
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Ratte
96 Tests Anmelden zum Anzeigen 13 bis 17 Tage
788,70 €
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Huhn
96 Tests Anmelden zum Anzeigen 13 bis 17 Tage
1.013,20 €
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Weitere ELISA Kits für LEPRE1 Interaktionspartner

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

  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 (zeige CRTAP ELISA Kits) 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 LEPRE1 knockout mouse is a valuable model system to investigate the mechanism of hearing loss in recessive osteogenesis imperfecta (zeige COL1A2 ELISA Kits).

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

  6. Study found that all 3 mouse Leprecan genes Lepre1, leprel1 (zeige LEPREL1 ELISA Kits) and leprel2 (zeige LEPREL2 ELISA Kits) are expressed in the notochord; additionally, they are expressed in dorsal root ganglia, limb buds, branchial arches, and developing kidneys.

  7. This report characterizes expression of the P3H1, P3H2 (zeige LEPREL1 ELISA Kits) and P3H3 (zeige LEPREL2 ELISA Kits) genes in embryonic and adult mice.

Human Leucine Proline-Enriched Proteoglycan (Leprecan) 1 (LEPRE1) Interaktionspartner

  1. P3H1 mutation is associated with Non-Lethal Type VIII (zeige COX8A ELISA Kits) Osteogenesis Imperfecta (zeige COL1A2 ELISA Kits).

  2. This study enhances our knowledge about the mutational pattern of the LEPRE1, CRTAP (zeige CRTAP ELISA Kits), and PPIB (zeige PPIB ELISA Kits) 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 (zeige COL1A2 ELISA Kits).

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

  7. We report a large consanguineous Turkish family in which multiple individuals are affected with autosomal recessive lethal or severe osteogenesis imperfecta (zeige COL1A2 ELISA Kits) (OI) due to a novel homozygous LEPRE1 mutation

  8. Null mutations in LEPRE1 and CRTAP (zeige CRTAP ELISA Kits) cause severe recessive osteogenesis imperfecta (zeige COL1A2 ELISA Kits).

  9. CRTAP (zeige CRTAP ELISA Kits) and P3H1 are mutually stabilized in the collagen prolyl 3-hydroxylation complex in endoplasmic reticulum.

  10. Screening of 78 subjects diagnosed with osteogenesis imperfecta (zeige COL1A2 ELISA Kits) type II or III, identified three probands with mutations in CRTAP (zeige CRTAP ELISA Kits) and 16 with mutations in LEPRE1.

LEPRE1 Antigen-Profil

Beschreibung des Gens

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.

Genbezeichner und Symbole assoziert mit LEPRE1

  • co-chaperonin GroES (groES) Antikörper
  • chaperonin GroS (groS1) Antikörper
  • 10 kDa chaperonin 1 (groS1) Antikörper
  • sb:cb953 (sb:cb953) Antikörper
  • leucine proline-enriched proteoglycan (leprecan) 1 (lepre1) Antikörper
  • leucine proline-enriched proteoglycan (leprecan) 1 (LEPRE1) Antikörper
  • leprecan 1 (Lepre1) Antikörper
  • leucine proline-enriched proteoglycan (leprecan) 1 (Lepre1) Antikörper
  • 2410024C15Rik Antikörper
  • GROS1 Antikörper
  • LEPRE1 Antikörper
  • MGC84556 Antikörper
  • OI8 Antikörper
  • P3H1 Antikörper
  • sb:cb953 Antikörper

Bezeichner auf Proteinebene für LEPRE1

co-chaperonin GroES , chaperonin GroS , 10 kDa chaperonin 1 , sb:cb953 , leucine proline-enriched proteoglycan (leprecan) 1 , leprecan 1 , prolyl 3-hydroxylase 1 , prolyl 3-hydroxylase 1-like , growth suppressor 1 , growth supressor 1 , leprecan-1 , leucine- and proline-enriched proteoglycan 1 , leprecan , leprecan-1 homolog , leucine- and proline-enriched proteoglycan 1 homolog

GENE ID SPEZIES
6933670 Burkholderia cenocepacia J2315
6941686 Thermodesulfovibrio yellowstonii DSM 11347
6988625 Aliivibrio salmonicida LFI1238
378992 Danio rerio
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
64175 Homo sapiens
114200 Rattus norvegicus
414142 Gallus gallus
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