Rabbit anti-Human PPT1 Polyclonal Antibody | anti-PPT1 antibody

PPT1, ID (PPT1, PPT, Palmitoyl-protein thioesterase 1, Palmitoyl-protein hydrolase 1)

Cat. Num. AAA6007999

• Gene Names: PPT1; PPT; CLN1; INCL
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: PPT1; Polyclonal Antibody; ID (PPT1; PPT; Palmitoyl-protein thioesterase 1; Palmitoyl-protein hydrolase 1); Anti -PPT1; anti-PPT1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

• Applicable Applications for anti-PPT1 antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in Western Blot, ELISA; Dilution: ELISA: 1:1,000; Western Blot: 1:100-500
• Immunogen: PPT1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 188-217 amino acids from the Central region of human PPT1.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(PPT1 Antibody (Center) western blot analysis in A549 cell line lysates (35ug/lane).This demonstrates the PPT1 antibody detected the PPT1 protein (arrow).)

Western Blot (WB)

(Western blot analysis of PPT1 (arrow) using rabbit polyclonal PPT1 Antibody (Center) . 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the PPT1 gene.)

Related Product Information for anti-PPT1 antibody

The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.

Product Categories/Family for anti-PPT1 antibody

Antibodies; Abs to Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 217272890
• NCBI GeneID: 5538
• NCBI Accession #: NP_001136076.1
• NCBI GenBank Nucleotide #: NM_001142604.1
• UniProt Accession #: P50897; Q6FGQ4; B4DY24
• Molecular Weight: 34,193 Da
• NCBI Official Full Name: palmitoyl-protein thioesterase 1 isoform 2
• NCBI Official Synonym Full Names: palmitoyl-protein thioesterase 1
• NCBI Official Symbol: PPT1
• NCBI Official Synonym Symbols: PPT; CLN1; INCL
• NCBI Protein Information: palmitoyl-protein thioesterase 1; palmitoyl-protein hydrolase 1; ceroid-palmitoyl-palmitoyl-protein thioesterase 1
• UniProt Protein Name: Palmitoyl-protein thioesterase 1
• Protein Family: Palmitoyl-protein thioesterase
• UniProt Gene Name: PPT1
• UniProt Synonym Gene Names: PPT; PPT-1
• UniProt Entry Name: PPT1_HUMAN

NCBI Description

The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Dec 2008]

Uniprot Description

PPT1: Removes thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons. Defects in PPT1 are the cause of neuronal ceroid lipofuscinosis type 1 (CLN1). A form of neuronal ceroid lipofuscinosis with variable age at onset. Infantile, late- infantile, juvenile, and adult onset have been reported. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN1 is referred to as granular osmiophilic deposits (GROD). Belongs to the palmitoyl-protein thioesterase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.1.2.22; Lipid Metabolism - fatty acid elongation in mitochondria; Hydrolase

Chromosomal Location of Human Ortholog: 1p32

Cellular Component: Golgi apparatus; extracellular space; synaptic vesicle; membrane; cell soma; axon; lysosome; dendrite; extracellular region; nucleus; cytosol; lipid raft

Molecular Function: palmitoyl-(protein) hydrolase activity; palmitoyl-CoA hydrolase activity

Biological Process: pinocytosis; adult locomotory behavior; neurotransmitter secretion; positive regulation of receptor-mediated endocytosis; protein transport; visual perception; protein catabolic process; negative regulation of neuron apoptosis; cofactor transport; associative learning; grooming behavior; nervous system development; receptor-mediated endocytosis; regulation of phospholipase A2 activity; response to stimulus; positive regulation of pinocytosis; sphingolipid catabolic process; cellular protein catabolic process; regulation of synapse structure and activity; lysosomal lumen acidification; neuron development; cofactor metabolic process; negative regulation of cell growth; brain development; lipid raft organization and biogenesis; lipid catabolic process; negative regulation of apoptosis; protein depalmitoylation

Disease: Ceroid Lipofuscinosis, Neuronal, 1