LEPRE1 Antikörper (AA 1-390)

Produktnummer ABIN528557

Produktdetails anti-LEPRE1 Antikörper

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

Bindungsspezifität: AA 1-390

Reaktivität: Human

Wirt: Maus

Klonalität: Polyklonal

Konjugat: Dieser LEPRE1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

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

Sequenz: 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

Kreuzreaktivität: Human

Produktmerkmale: Antibody reactive against mammalian transfected lysate.

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

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Buffer: In 1x PBS, pH 7.4

Handhabung: Aliquot to avoid repeated freezing and thawing.

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.

Referenzen für anti-LEPRE1 Antikörper (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).

Details zu LEPRE1

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

Andere Bezeichnung: LEPRE1 (LEPRE1 Produkte)

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

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

Gen-ID: 64175