推荐产品
生物来源
rabbit
质量水平
偶联物
unconjugated
抗体形式
purified immunoglobulin
抗体产品类型
primary antibodies
克隆
polyclonal
表单
buffered aqueous solution
分子量
antigen 36.5 kDa
种属反应性
human
技术
western blot: 1 μg/mL
NCBI登记号
UniProt登记号
运输
dry ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... RLBP1(6017)
一般描述
Retinaldehyde binding protein 1 (RLBP1) mapped to human chromosome 15q26, encodes cellular retinaldehyde-binding protein (CRALBP).1 RLBP1 is a 36kDa water soluble protein and is expressed exclusively in retina and pineal gland.
The protein encoded by this gene is a 36kDa water-soluble protein which carries 11-cis-retinaldehyde or 11-cis-retinal as physiologic ligands. It may be a functional component of the visual cycle. Mutations of this gene have been associated with severe rod-cone dystrophy, Bothnia dystrophy (nonsyndromic autosomal recessive retinitis pigmentosa) and retinitis punctata albescens. (provided by RefSeq)
免疫原
RLBP1 (NP_000317.1, 1 a.a. ~ 317 a.a) full-length human protein.
Sequence
MSEGVGTFRMVPEEEQELRAQLEQLTTKDHGPVFGPCSQLPRHTLQKAKDELNEREETREEAVRELQEMVQAQAASGEELAVAVAERVQEKDSGFFLRFIRARKFNVGRAYELLRGYVNFRLQYPELFDSLSPEAVRCTIEAGYPGVLSSRDKYGRVVMLFNIENWQSQEITFDEILQAYCFILEKLLENEETQINGFCIIENFKGFTMQQAASLRTSDLRKMVDMLQDSFPARFKAIHFIHQPWYFTTTYNVVKPFLKSKLLERVFVHGDDLSGFYQEIDENILPSDFGGTLPKYDGKAVAEQLFGPQAQAENTAF
Sequence
MSEGVGTFRMVPEEEQELRAQLEQLTTKDHGPVFGPCSQLPRHTLQKAKDELNEREETREEAVRELQEMVQAQAASGEELAVAVAERVQEKDSGFFLRFIRARKFNVGRAYELLRGYVNFRLQYPELFDSLSPEAVRCTIEAGYPGVLSSRDKYGRVVMLFNIENWQSQEITFDEILQAYCFILEKLLENEETQINGFCIIENFKGFTMQQAASLRTSDLRKMVDMLQDSFPARFKAIHFIHQPWYFTTTYNVVKPFLKSKLLERVFVHGDDLSGFYQEIDENILPSDFGGTLPKYDGKAVAEQLFGPQAQAENTAF
生化/生理作用
Retinaldehyde binding protein 1 (RLBP1) plays a vital role in mammalian vision by directing 11-cis-retinoids for the conversion of photobleached opsin molecules into photosensitive visual pigments. Genetic variations in the gene leads to various autosomal recessive progressive retinopathy, including Bothnia dystrophy (BD) and retinitis, punctata albescens (RPA), Newfoundland rod-cone dystrophy (NFRCD), retinitis pigmentosa (RP) and fundus albipunctatus (FA).
外形
Solution in phosphate buffered saline, pH 7.4
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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储存分类代码
10 - Combustible liquids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
新产品
Elodie Dessalces et al.
JAMA ophthalmology, 131(10), 1314-1323 (2013-08-10)
Retinitis punctata albescens (RPA) is an autosomal recessive form of retinitis pigmentosa characterized by white dotlike deposits in the fundus, in most cases caused by mutations in RLBP1. To study disease progression and visual function in RPA. We performed clinical
Xiaoqin He et al.
Proceedings of the National Academy of Sciences of the United States of America, 106(44), 18545-18550 (2009-10-23)
Cellular retinaldehyde-binding protein (CRALBP) is essential for mammalian vision by routing 11-cis-retinoids for the conversion of photobleached opsin molecules into photosensitive visual pigments. The arginine-to-tryptophan missense mutation in position 234 (R234W) in the human gene RLBP1 encoding CRALBP compromises visual
Stephanie Hipp et al.
Acta ophthalmologica, 93(4), e281-e286 (2014-11-29)
Mutations in the RLBP1 gene encoding the cellular retinaldehyde-binding protein (CRALBP) cause autosomal recessive progressive retinopathy, such as retinitis punctata albescens (RPA), Bothnia-type dystrophy (BD), Newfoundland rod-cone dystrophy (NFRCD), retinitis pigmentosa (RP) and fundus albipunctatus (FA). We present the clinical
Molecular clues to Bothnia-type retinal dystrophy.
Xiaoqin He et al.
Advances in experimental medicine and biology, 723, 589-594 (2011-12-21)
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