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SAB1411057

Sigma-Aldrich

Anti-PPT1 antibody produced in rabbit

purified immunoglobulin, buffered aqueous solution

Synonym(s):

CLN1, INCL, PPT

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

rabbit

Quality Level

100

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.

NM_000310

UniProt accession no.

P50897

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... PPT1(5538)

Related Categories

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

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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wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

Certificates of Analysis (COA)

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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)
Reversible cysteine acylation regulates the activity of human palmitoyl-protein thioesterase 1 (PPT1)
Segal-Salto M, et al.
Testing, 11(1), e0146466-e0146466 (2016)

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