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 34.2 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 ... PPT1(5538)
一般描述
Palmitoyl-protein thioesterase 1 (PPT1) is a lysosomal enzyme 1) It is mapped to human chromosome 1p34.2 and has eight exons. It is depalmitoylation enzyme with a signal sequence and is targeted to lysosomes through mannose 6-phosphate receptor pathway. It is also localized in axons, synaptic vesicles and synaptosomes of neurons.
免疫原
PPT1 (NP_000301.1, 1 a.a. ~ 306 a.a) full-length human protein.
Sequence
MASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLSMGAIKKMVEKKIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALAKDPKLQQGYNAMGFSQGGQFLRAVAQRCPSPPMINLISVGGQHQGVFGLPRCPGESSHICDFIRKTLNAGAYSKVVQERLVQAEYWHDPIKEDVYRNHSIFLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAHIIPFLG
Sequence
MASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLSMGAIKKMVEKKIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALAKDPKLQQGYNAMGFSQGGQFLRAVAQRCPSPPMINLISVGGQHQGVFGLPRCPGESSHICDFIRKTLNAGAYSKVVQERLVQAEYWHDPIKEDVYRNHSIFLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAHIIPFLG
生化/生理作用
Palmitoyl-protein thioesterase 1 (PPT1) functions to remove fatty acid associated with lipid-modified proteins from the cysteine residues. Mutations in the CLN1 or the PPT1 gene is implicated in the cognitive and motor decline disorder in infants called the infantile neuronal ceroid lipofuscinosis. PPT1 is glycosylated and is essential for its functionality.
外形
Solution in phosphate buffered saline, pH 7.4
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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
Reversible cysteine acylation regulates the activity of human palmitoyl-protein thioesterase 1 (PPT1)
Segal-Salto M, et al.
Testing, 11(1), e0146466-e0146466 (2016)
Experimental therapies in the neuronal ceroid lipofuscinoses
Neverman NJ, et al.
Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease, 1852(10), 2292-2300 (2015)
Structural basis of neuronal ceroid lipofuscinosis 1
Ohno K, et al.
Brain & Development, 32(7), 524-530 (2010)
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