LEPRE1 产品
(Leucine Proline-Enriched Proteoglycan (Leprecan) 1 (LEPRE1))
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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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Featured LEPRE1 Categories
LEPRE1 抗体
High quality antibodies with extensive validation data.
LEPRE1 ELISA试剂盒
Reliable ELISA kits for a wide range of species.
LEPRE1 蛋白
Proteins for various applications incl. WB, ELISA, IF etc.
Recommended LEPRE1 抗体
Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity
Human
Application
WB, ELISA, IF, IHC (p)
Validations
- (4)
- (6)
Cat. No.
ABIN566242
Quantity
100 μg
Datasheet
Datasheet
Reactivity
Human
Application
WB
Validations
- (9)
- (2)
Cat. No.
ABIN528557
Quantity
50 μg
Datasheet
Datasheet
Reactivity
Human
Application
WB, FACS, IF
Validations
- (3)
Cat. No.
ABIN1499128
Quantity
100 μL
Datasheet
Datasheet
Recommended LEPRE1 ELISA试剂盒
Product
Reactivity
Analytical Method
Validations
Cat. No.
Quantity
Datasheet
Reactivity
Human
Analytical Method
Quantitative Sandwich ELISA
Validations
- (1)
Cat. No.
ABIN6968262
Quantity
96 tests
Datasheet
Datasheet
Reactivity
Human
Analytical Method
Quantitative Sandwich ELISA
Validations
- (1)
Cat. No.
ABIN6957446
Quantity
96 tests
Datasheet
Datasheet
Reactivity
Mouse
Analytical Method
Validations
Cat. No.
ABIN1150181
Quantity
96 tests
Datasheet
Datasheet
Recommended LEPRE1 蛋白
Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity
Human
Source
Wheat germ
Validations
- (1)
Cat. No.
ABIN1309311
Quantity
10 μg
Datasheet
Datasheet
Reactivity
Human
Source
Wheat germ
Validations
- (1)
Cat. No.
ABIN1309313
Quantity
10 μg
Datasheet
Datasheet
Reactivity
Human
Source
HEK-293 Cells
Validations
- (1)
Cat. No.
ABIN2724652
Quantity
20 μg
Datasheet
Datasheet
Latest Publications for our LEPRE1 Products
: "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).
Synonyms and alternative names related to 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:cb953Protein 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
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