Goat anti-Npc1(mouse) Antibody | MyBioSource MBS422388 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Goat anti-Npc1(mouse) Antibody

catalog :

MBS422388

quantity :

0.1 mg

price :

225 USD

clonality :

polyclonal

host :

goat

conjugate :

nonconjugated

reactivity :

mouse

application :

ELISA, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS422388

products type :

Antibody

products full name :

Goat anti-Npc1(mouse) Antibody

products short name :

Npc1

products name syn :

Npc1; Niemann Pick type C1; A430089E03Rik; C85354; D18Ertd139e; D18Ertd723e; lcsd; nmf164; spm; niemann-Pick C1 protein; sphingomyelinosis; A430089E03Rik antibody; C85354 antibody; D18Ertd139e antibody; D18Ertd723e antibody; lcsd antibody; Niemann Pick type C1 antibody; niemann-Pick C1 protein antibody; nmf164 antibody; sphingomyelinosis antibody; spm antibody; Npc1 antibody; Npc1(mouse)

other names :

Niemann-Pick C1 protein; Niemann-Pick C1 protein; Niemann-Pick C1 protein; Niemann-Pick type C1

products gene name :

Npc1

other gene names :

Npc1; Npc1; spm; lcsd; C85354; nmf164; D18Ertd139e; D18Ertd723e; A430089E03Rik

uniprot entry name :

NPC1_MOUSE

clonality :

Polyclonal

host :

Goat

reactivity :

Expected from sequence similarity: Mouse

sequence length :

1277

sequence :

TYHTILKTSADYTD

purity :

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

form :

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

concentration :

100ug specific antibody in 200ul

storage stability :

Aliquot and store at -20 degree C. Minimize freezing and thawing.

tested application :

Peptide ELISA (EIA)

app notes :

Peptide ELISA: Antibody detection limit dilution 1: 16000. Western Blot: Preliminary experiments gave an approx 36kDa band in lysates of cell lines NIH3T3 and NSO after 1ug/ml antibody staining. Please note that currently we cannot find an explanation in the literature for the band we observe given the calculated size of 142kDa according to NP_032746.2. The 36kDa band was successfully blocked by incubation with the immunizing peptide.

other info1 :

Immunogen: Peptide with sequence C-TYHTILKTSADYTD, from the internal region of the protein sequence according to NP_032746.2. Epitope: Internal region

ncbi gi num :

89242146

ncbi acc num :

NP_032746.2

ncbi gb acc num :

NM_008720.2

ncbi mol weight :

142,885 Da

ncbi pathways :

Lysosome Pathway (99272); Lysosome Pathway (96865)

uniprot summary :

NPC1: Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals. Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPC1). A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C1 has a highly variable clinical phenotype. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood. An allelic variant of Niemann-Pick disease type C1 is found in people with Nova Scotia ancestry. Patients with the Nova Scotian clinical variant are less severely affected. Belongs to the patched family. Protein type: Membrane protein, integral; Membrane protein, multi-pass. Cellular Component: endoplasmic reticulum; endosome; extracellular region; Golgi apparatus; integral to membrane; integral to plasma membrane; lipid raft; lysosomal membrane; lysosome; membrane; nuclear envelope; perinuclear region of cytoplasm; plasma membrane; vesicle. Molecular Function: cholesterol binding; hedgehog receptor activity; lipid transporter activity; protein binding. Biological Process: adult walking behavior; autophagy; cholesterol efflux; cholesterol homeostasis; cholesterol metabolic process; cholesterol transport; endocytosis; entry of virus into host cell; lipid metabolic process; lipid raft organization and biogenesis; negative regulation of macroautophagy; protein amino acid glycosylation; response to cadmium ion; response to drug; steroid metabolic process

size1 :

0.1 mg

price1 :

225 USD

more info or order :

MyBioSource product webpage