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Western blotAntibody data
- Antibody Data
- Antigen structure
- References [8]
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- Validations
- Western blot [2]
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- Product number
- H00064175-B01P - Provider product page
- Provider
- Abnova Corporation
- Proper citation
- Abnova Corporation Cat#H00064175-B01P, RRID:AB_1137611
- Product name
- LEPRE1 purified MaxPab mouse polyclonal antibody (B01P)
- Antibody type
- Polyclonal
- Description
- Mouse polyclonal antibody raised against a full-length human LEPRE1 protein.
- Antigen sequence
MLGEEHTRSIGPRESAKEYRQRSLLEKELLFFAYD
VFGIPFVDPDSWTPEEVIPKRLQEKQKSERETAVR
ISQEIGNLMKEIETLVEEKTKESLDVSRLTREGGP
LLYEGISLTMNSKLLNGSQRVVMDGVISDHECQEL
QRLTNVAATSGDGYRGQTSPHTPNEKFYGVTVFKA
LKLGQEGKVPLQSAHLYYNVTEKVRRIMESYFRLD
TPLYFSYSHLVCRTAIEEVQAERKDDSHPVHVDNC
ILNAETLVCVKEPPAYTFRDYSAILYLNGDFDGGN
FYFTELDAKTVTAEVQPQCGRAVGFSSGTENPHGV
KAVTRGQRCAIALWFTLDPRHSERDRVQADDLVKM
LFSPEEMDLSQEQPLDAQQGPPEPAQESLSGSESK
PKDEL- Storage
- Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
Submitted references Abnormal type I collagen post-translational modification and crosslinking in a cyclophilin B KO mouse model of recessive osteogenesis imperfecta.
A novel mutation in LEPRE1 that eliminates only the KDEL ER- retrieval sequence causes non-lethal osteogenesis imperfecta.
Severe osteogenesis imperfecta caused by a small in-frame deletion in CRTAP.
Mutations in PPIB (cyclophilin B) delay type I procollagen chain association and result in perinatal lethal to moderate osteogenesis imperfecta phenotypes.
Generalized connective tissue disease in Crtap-/- mouse.
Prolyl 3-hydroxylase 1 and CRTAP are mutually stabilizing in the endoplasmic reticulum collagen prolyl 3-hydroxylation complex.
PPIB mutations cause severe osteogenesis imperfecta.
Recessive osteogenesis imperfecta caused by LEPRE1 mutations: clinical documentation and identification of the splice form responsible for prolyl 3-hydroxylation.
Cabral WA, Perdivara I, Weis M, Terajima M, Blissett AR, Chang W, Perosky JE, Makareeva EN, Mertz EL, Leikin S, Tomer KB, Kozloff KM, Eyre DR, Yamauchi M, Marini JC
PLoS genetics 2014 Jun;10(6):e1004465
PLoS genetics 2014 Jun;10(6):e1004465
A novel mutation in LEPRE1 that eliminates only the KDEL ER- retrieval sequence causes non-lethal osteogenesis imperfecta.
Takagi M, Ishii T, Barnes AM, Weis M, Amano N, Tanaka M, Fukuzawa R, Nishimura G, Eyre DR, Marini JC, Hasegawa T
PloS one 2012;7(5):e36809
PloS one 2012;7(5):e36809
Severe osteogenesis imperfecta caused by a small in-frame deletion in CRTAP.
Amor IM, Rauch F, Gruenwald K, Weis M, Eyre DR, Roughley P, Glorieux FH, Morello R
American journal of medical genetics. Part A 2011 Nov;155A(11):2865-70
American journal of medical genetics. Part A 2011 Nov;155A(11):2865-70
Mutations in PPIB (cyclophilin B) delay type I procollagen chain association and result in perinatal lethal to moderate osteogenesis imperfecta phenotypes.
Pyott SM, Schwarze U, Christiansen HE, Pepin MG, Leistritz DF, Dineen R, Harris C, Burton BK, Angle B, Kim K, Sussman MD, Weis M, Eyre DR, Russell DW, McCarthy KJ, Steiner RD, Byers PH
Human molecular genetics 2011 Apr 15;20(8):1595-609
Human molecular genetics 2011 Apr 15;20(8):1595-609
Generalized connective tissue disease in Crtap-/- mouse.
Baldridge D, Lennington J, Weis M, Homan EP, Jiang MM, Munivez E, Keene DR, Hogue WR, Pyott S, Byers PH, Krakow D, Cohn DH, Eyre DR, Lee B, Morello R
PloS one 2010 May 11;5(5):e10560
PloS one 2010 May 11;5(5):e10560
Prolyl 3-hydroxylase 1 and CRTAP are mutually stabilizing in the endoplasmic reticulum collagen prolyl 3-hydroxylation complex.
Chang W, Barnes AM, Cabral WA, Bodurtha JN, Marini JC
Human molecular genetics 2010 Jan 15;19(2):223-34
Human molecular genetics 2010 Jan 15;19(2):223-34
PPIB mutations cause severe osteogenesis imperfecta.
van Dijk FS, Nesbitt IM, Zwikstra EH, Nikkels PG, Piersma SR, Fratantoni SA, Jimenez CR, Huizer M, Morsman AC, Cobben JM, van Roij MH, Elting MW, Verbeke JI, Wijnaendts LC, Shaw NJ, Högler W, McKeown C, Sistermans EA, Dalton A, Meijers-Heijboer H, Pals G
American journal of human genetics 2009 Oct;85(4):521-7
American journal of human genetics 2009 Oct;85(4):521-7
Recessive osteogenesis imperfecta caused by LEPRE1 mutations: clinical documentation and identification of the splice form responsible for prolyl 3-hydroxylation.
Willaert A, Malfait F, Symoens S, Gevaert K, Kayserili H, Megarbane A, Mortier G, Leroy JG, Coucke PJ, De Paepe A
Journal of medical genetics 2009 Apr;46(4):233-41
Journal of medical genetics 2009 Apr;46(4):233-41
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Supportive validation
- Submitted by
- Abnova Corporation (provider)
- Main image
- Experimental details
- LEPRE1 MaxPab polyclonal antibody. Western Blot analysis of LEPRE1 expression in human placenta.
- Submitted by
- Abnova Corporation (provider)
- Main image
- Experimental details
- Western Blot analysis of LEPRE1 expression in transfected 293T cell line (H00064175-T01) by LEPRE1 MaxPab polyclonal antibody.Lane 1: LEPRE1 transfected lysate(42.9 KDa).Lane 2: Non-transfected lysate.
