catalog number :
MBS9317749
products type :
ELISA Kit
products full name :
Human Palmitoyl-protein thioesterase 1, PPT1 ELISA Kit
products short name :
[palmitoyl-protein thioesterase 1]
products name syn :
[Human Palmitoyl-protein thioesterase 1 (PPT1) ELISA kit; RP11-115D7.2; CLN1; INCL; PPT; ceroid-palmitoyl-palmitoyl-protein thioesterase 1; palmitoyl-protein hydrolase 1; palmitoyl-protein thioesterase 1]
other names :
[palmitoyl-protein thioesterase 1 isoform 1; Palmitoyl-protein thioesterase 1; palmitoyl-protein thioesterase 1; palmitoyl-protein hydrolase 1; ceroid-palmitoyl-palmitoyl-protein thioesterase 1; palmitoyl-protein thioesterase 1; Palmitoyl-protein hydrolase 1]
products gene name :
[PPT1]
other gene names :
[PPT1; PPT1; PPT; CLN1; INCL; PPT; PPT-1]
uniprot entry name :
PPT1_HUMAN
specificity :
No significant cross-reactivity or interference between this analyte and analogues is observed.
storage stability :
Store all reagents at 2-8 degree C
other info1 :
Samples: Rabbit Body Fluids And Tissue Homogenates. Assay Type: Quantitative Sandwich. Detection Range: 0.25ng/ml-8ng/ml. Sensitivity: 0.1ng/ml.
other info2 :
Reactivity Note: Rabbit reactivity is predicted based on homology with both the human and rat PPP2R4 sequence and also positive reference data for rabbit samples with this assay. Please inquire for additional information. Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100]. All CV% should be compared by concentration, not compared by OD values.
products description :
Background/Introduction: This Quantitative Sandwich ELISA kit is for lab reagent/research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determine the level of PPP2R4 (hereafter termed "analyte") in undiluted original Rabbit body fluids and tissue homogenates. This kit is NOT suitable for assaying non-biological sources of substances.
ncbi acc num :
NP_000301.1
ncbi gb acc num :
NM_000310.3
ncbi mol weight :
34,193 Da
ncbi pathways :
Fatty Acid Elongation Pathway (82934); Fatty Acid Elongation Pathway (295); Lysosome Pathway (99052); Lysosome Pathway (96865)
ncbi summary :
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 summary :
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: Lipid Metabolism - fatty acid elongation in mitochondria; EC 3.1.2.22; Hydrolase. Chromosomal Location of Human Ortholog: 1p32. Cellular Component: Golgi apparatus; extracellular space; synaptic vesicle; cell soma; membrane; 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; cofactor transport; negative regulation of neuron apoptosis; 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; brain development; negative regulation of cell growth; lipid raft organization and biogenesis; lipid catabolic process; negative regulation of apoptosis; protein depalmitoylation. Disease: Ceroid Lipofuscinosis, Neuronal, 1
size4 :
10x96-Strip-Wells