LEPRE1 Produkte
(Leucine Proline-Enriched Proteoglycan (Leprecan) 1 (LEPRE1))
Kategorien
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].
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Ausgewählte LEPRE1 Kategorien
LEPRE1 Antikörper
High quality antibodies with extensive validation data.
LEPRE1 ELISA Kits
Reliable ELISA kits for a wide range of species.
LEPRE1 Proteine
Proteins for various applications incl. WB, ELISA, IF etc.
Empfohlene LEPRE1 Antikörper
Produkt
Reaktivität
Applikation
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität
Human
Applikation
WB, ELISA, IF, IHC (p)
Validierungen
- (4)
- (6)
Kat. Nr.
ABIN566242
Menge
100 μg
Datenblatt
Datenblatt
Reaktivität
Human
Applikation
WB
Validierungen
- (9)
- (2)
Kat. Nr.
ABIN528557
Menge
50 μg
Datenblatt
Datenblatt
Reaktivität
Human
Applikation
WB, FACS, IF
Validierungen
- (3)
Kat. Nr.
ABIN1499128
Menge
100 μL
Datenblatt
Datenblatt
Empfohlene LEPRE1 ELISA Kits
Produkt
Reaktivität
Analytical Method
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität
Human
Analytical Method
Quantitative Sandwich ELISA
Validierungen
- (1)
Kat. Nr.
ABIN6968262
Menge
96 tests
Datenblatt
Datenblatt
Reaktivität
Human
Analytical Method
Quantitative Sandwich ELISA
Validierungen
- (1)
Kat. Nr.
ABIN6957446
Menge
96 tests
Datenblatt
Datenblatt
Reaktivität
Mouse
Analytical Method
Validierungen
Kat. Nr.
ABIN1150181
Menge
96 tests
Datenblatt
Datenblatt
Empfohlene LEPRE1 Proteine
Produkt
Reaktivität
Source
Validierungen
Kat. Nr.
Menge
Datenblatt
Reaktivität
Human
Source
Wheat germ
Validierungen
- (1)
Kat. Nr.
ABIN1309311
Menge
10 μg
Datenblatt
Datenblatt
Reaktivität
Human
Source
Wheat germ
Validierungen
- (1)
Kat. Nr.
ABIN1309313
Menge
10 μg
Datenblatt
Datenblatt
Reaktivität
Human
Source
HEK-293 Cells
Validierungen
- (1)
Kat. Nr.
ABIN2724652
Menge
20 μg
Datenblatt
Datenblatt
Neueste Publikationen zu unseren LEPRE1 Produkten
: "Targeted exome sequencing identifies novel compound heterozygous mutations in P3H1 in a fetus with osteogenesis imperfecta type VIII." in: Clinica chimica acta; international journal of clinical chemistry, Vol. 464, pp. 170-175, (2016) (PubMed).: "Localization of collagen modifying enzymes on fibroblastic reticular cells and follicular dendritic cells in non-neoplastic and neoplastic lymphoid tissues." in: Leukemia & lymphoma, Vol. 57, Issue 7, pp. 1687-96, (2016) (PubMed).
: "Abnormal type I collagen post-translational modification and crosslinking in a cyclophilin B KO mouse model of recessive osteogenesis imperfecta." in: PLoS genetics, Vol. 10, Issue 6, pp. e1004465, (2014) (PubMed).
: "A novel mutation in LEPRE1 that eliminates only the KDEL ER- retrieval sequence causes non-lethal osteogenesis imperfecta." in: PLoS ONE, Vol. 7, Issue 5, pp. e36809, (2012) (PubMed).
: "Mutations in PPIB (cyclophilin B) delay type I procollagen chain association and result in perinatal lethal to moderate osteogenesis imperfecta phenotypes." in: Human molecular genetics, Vol. 20, Issue 8, pp. 1595-609, (2011) (PubMed).
: "Severe osteogenesis imperfecta caused by a small in-frame deletion in CRTAP." in: American journal of medical genetics. Part A, Vol. 155A, Issue 11, pp. 2865-70, (2011) (PubMed).
: "Proteomic dissection of the von Hippel-Lindau (VHL) interactome." in: Journal of proteome research, Vol. 10, Issue 11, pp. 5175-82, (2011) (PubMed).
: "Generalized connective tissue disease in Crtap-/- mouse." in: PLoS ONE, Vol. 5, Issue 5, pp. e10560, (2010) (PubMed).
: "Recessive osteogenesis imperfecta caused by LEPRE1 mutations: clinical documentation and identification of the splice form responsible for prolyl 3-hydroxylation." in: Journal of medical genetics, Vol. 46, Issue 4, pp. 233-41, (2009) (PubMed).
: "PPIB mutations cause severe osteogenesis imperfecta." in: American journal of human genetics, Vol. 85, Issue 4, pp. 521-7, (2009) (PubMed).
Synonyme und alternative Namen zu LEPRE1
prolyl 3-hydroxylase 1 (P3H1), prolyl 3-hydroxylase 1 (P3h1), prolyl 3-hydroxylase 1 L homeolog (p3h1.L), prolyl 3-hydroxylase 1 (p3h1), 2410024C15Rik, GROS1, Gros1, LEPRE1, MGC84556, OI8, P3H1, sb:cb953Bezeichner auf Proteinebene für 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
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