LEPRE1 antibody (AA 1-390)

Catalog No. ABIN528557

Product Details anti-LEPRE1 Antibody

Target: LEPRE1 (Leucine Proline-Enriched Proteoglycan (Leprecan) 1 (LEPRE1))

Binding Specificity: AA 1-390

Reactivity: Human

Host: Mouse

Clonality: Polyclonal

Conjugate: This LEPRE1 antibody is un-conjugated

Application: Western Blotting (WB)

Purpose: Mouse polyclonal antibody raised against a full-length human LEPRE1 protein.

Sequence: MLGEEHTRSI GPRESAKEYR QRSLLEKELL FFAYDVFGIP FVDPDSWTPE EVIPKRLQEK QKSERETAVR ISQEIGNLMK EIETLVEEKT KESLDVSRLT REGGPLLYEG ISLTMNSKLL NGSQRVVMDG VISDHECQEL QRLTNVAATS GDGYRGQTSP HTPNEKFYGV TVFKALKLGQ EGKVPLQSAH LYYNVTEKVR RIMESYFRLD TPLYFSYSHL VCRTAIEEVQ AERKDDSHPV HVDNCILNAE TLVCVKEPPA YTFRDYSAIL YLNGDFDGGN FYFTELDAKT VTAEVQPQCG RAVGFSSGTE NPHGVKAVTR GQRCAIALWF TLDPRHSERD RVQADDLVKM LFSPEEMDLS QEQPLDAQQG PPEPAQESLS GSESKPKDEL

Cross-Reactivity: Human

Characteristics: Antibody reactive against mammalian transfected lysate.

Immunogen: LEPRE1 (AAH15309, 1 a.a. ~ 390 a.a) full-length human protein.

Application Details

Application Notes: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Handling

Buffer: In 1x PBS, pH 7.4

Handling Advice: Aliquot to avoid repeated freezing and thawing.

Storage: -20 °C

Storage Comment: Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.

References for anti-LEPRE1 antibody (ABIN528557)

Huang, Mei, Lv, Li, Zhang, Pan, Tan, Guo, Luo, Chen, Liang, Wu: "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).

Cabral, Perdivara, Weis, Terajima, Blissett, Chang, Perosky, Makareeva, Mertz, Leikin, Tomer, Kozloff, Eyre, Yamauchi, Marini: "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).

Takagi, Ishii, Barnes, Weis, Amano, Tanaka, Fukuzawa, Nishimura, Eyre, Marini, Hasegawa: "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).

Pyott, Schwarze, Christiansen, Pepin, Leistritz, Dineen, Harris, Burton, Angle, Kim, Sussman, Weis, Eyre, Russell, McCarthy, Steiner, Byers: "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).

Amor, Rauch, Gruenwald, Weis, Eyre, Roughley, Glorieux, Morello: "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).

Baldridge, Lennington, Weis, Homan, Jiang, Munivez, Keene, Hogue, Pyott, Byers, Krakow, Cohn, Eyre, Lee, Morello: "Generalized connective tissue disease in Crtap-/- mouse." in: PLoS ONE, Vol. 5, Issue 5, pp. e10560, (2010) (PubMed).

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." in: Journal of medical genetics, Vol. 46, Issue 4, pp. 233-41, (2009) (PubMed).

van Dijk, Nesbitt, Zwikstra, Nikkels, Piersma, Fratantoni, Jimenez, Huizer, Morsman, Cobben, van Roij, Elting, Verbeke, Wijnaendts, Shaw, Högler, McKeown, Sistermans, Dalton, Meijers-Heijboer, Pals: "PPIB mutations cause severe osteogenesis imperfecta." in: American journal of human genetics, Vol. 85, Issue 4, pp. 521-7, (2009) (PubMed).

Chang, Barnes, Cabral, Bodurtha, Marini: "Prolyl 3-hydroxylase 1 and CRTAP are mutually stabilizing in the endoplasmic reticulum collagen prolyl 3-hydroxylation complex." in: Human molecular genetics, Vol. 19, Issue 2, pp. 223-34, (2009) (PubMed).

Target Details for LEPRE1

Target: LEPRE1 (Leucine Proline-Enriched Proteoglycan (Leprecan) 1 (LEPRE1))

Alternative Name: LEPRE1 (LEPRE1 Products)

Synonyms: GROS1 antibody, OI8 antibody, P3H1 antibody, Gros1 antibody, MGC84556 antibody, LEPRE1 antibody, sb:cb953 antibody, 2410024C15Rik antibody, prolyl 3-hydroxylase 1 antibody, prolyl 3-hydroxylase 1 L homeolog antibody, P3H1 antibody, P3h1 antibody, p3h1.L antibody, p3h1 antibody

Background: Full Gene Name: leucine proline-enriched proteoglycan (leprecan) 1
Synonyms: GROS1,MGC117314,P3H1

Gene ID: 64175