SAB1411057
Anti-PPT1 antibody produced in rabbit
purified immunoglobulin, buffered aqueous solution
别名:
CLN1, INCL, PPT
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关于此项目
NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
WB
Citations:
3
biological source
rabbit
conjugate
unconjugated
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
antigen 34.2 kDa
species reactivity
human
technique(s)
western blot: 1 μg/mL
NCBI accession no.
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... PPT1(5538)
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General description
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.
Immunogen
PPT1 (NP_000301.1, 1 a.a. ~ 306 a.a) full-length human protein.
Sequence
MASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLSMGAIKKMVEKKIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALAKDPKLQQGYNAMGFSQGGQFLRAVAQRCPSPPMINLISVGGQHQGVFGLPRCPGESSHICDFIRKTLNAGAYSKVVQERLVQAEYWHDPIKEDVYRNHSIFLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAHIIPFLG
Sequence
MASPGCLWLLAVALLPWTCASRALQHLDPPAPLPLVIWHGMGDSCCNPLSMGAIKKMVEKKIPGIYVLSLEIGKTLMEDVENSFFLNVNSQVTTVCQALAKDPKLQQGYNAMGFSQGGQFLRAVAQRCPSPPMINLISVGGQHQGVFGLPRCPGESSHICDFIRKTLNAGAYSKVVQERLVQAEYWHDPIKEDVYRNHSIFLADINQERGINESYKKNLMALKKFVMVKFLNDSIVDPVDSEWFGFYRSGQAKETIPLQETSLYTQDRLGLKEMDNAGQLVFLATEGDHLQLSEEWFYAHIIPFLG
Biochem/physiol Actions
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.
Physical form
Solution in phosphate buffered saline, pH 7.4
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存储类别
10 - Combustible liquids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
新产品
此项目有
Reversible cysteine acylation regulates the activity of human palmitoyl-protein thioesterase 1 (PPT1)
Segal-Salto M, et al.
Testing, 11(1), e0146466-e0146466 (2016)
Structural basis of neuronal ceroid lipofuscinosis 1
Ohno K, et al.
Brain & Development, 32(7), 524-530 (2010)
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)
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