Welcome to Omnimabs!
TEL: 02154156726 Info: info@Omnimabs.com CN

Niemann Pick C1 [JB87-33]

Catalog Number:OM629153
OM629153
Discount Price
OM629153-100ul
1~2 Week
100ul
OM629153-50ul
50ul
OM629153-20ul
20ul
1~2 Week
Product Profile
Product Name Niemann Pick C1 [JB87-33]
Antibody Type Primary Antibodies
Antigen Alias Niemann Pick C1 protein precursor antibody Niemann Pick disease, type C1 antibody Niemann-Pick C1 protein antibody NPC antibody NPC1 antibody NPC1_HUMAN antibody
Product description Cells obtain cholesterol via two distinct pathways, endogenous synthesis in the endoplasmic reticulum and exogenous uptake through the low-density lipoprotein (LDL) receptor pathway. NPC1 is a protein that resides in late endosomes and lysosomes and is involved in the intracellular trafficking of cholesterol. The human NPC1 gene maps to chromosome 18q11.2 and produces proteins which undergo N-glycosylation and are expressed in brain and liver. NPC1 contains a cysteine-rich domain, which is critical for proper protein function, but is highly mutated. Mutations in NPC1 result in Niemann-Pick disease type C (NPC), an autosomal recessive disease characterized by the accumulation of unesterified cholesterol in the endosomal/lysosomal system. The accumulation of cholesterol results in progressive neurodegeneration and death. More than 90% of cases of NPC are due to mutations in NPC1 and patients with NPC display multiple neurological symptoms, such as hepatosplenomegaly, ataxia, dystonia and dementia.
Immunogen Recombinant protein
Key Feature
Clonality Monoclonal
Isotype IgG
Host Species Recombinant rabbit
Tested Applications WBICCIFIHCFC
WB:1:500-1:1,000
ICC/IF:1:50-1:200
IHC:1:50-1:200
FC:1:50-1:100
Species Reactivity HumanMouseRat
Concentration 1mg/ml
Target Information
Alternative Names Niemann Pick C1 protein precursor antibody Niemann Pick disease
type C1 antibody Niemann-Pick C1 protein antibody NPC antibody NPC1 antibody NPC1_HUMAN antibody
Molecular Weight(MW) 142 kDa
Cellular Localization Endosome. Lysosome.
Database Links
SwissProt ID O15118 O35604
Application

Application

Fig8: Flow cytometric analysis of SH-SY-5Y cells with Niemann Pick C1 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black). Alexa Fluor 488-conjugated goat anti rabbit IgG was used as the secondary antibody.

Application

Fig8: Flow cytometric analysis of SH-SY-5Y cells with Niemann Pick C1 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black). Alexa Fluor 488-conjugated goat anti rabbit IgG was used as the secondary antibody.

Application

Fig8: Flow cytometric analysis of SH-SY-5Y cells with Niemann Pick C1 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black). Alexa Fluor 488-conjugated goat anti rabbit IgG was used as the secondary antibody.

Application

Fig8: Flow cytometric analysis of SH-SY-5Y cells with Niemann Pick C1 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black). Alexa Fluor 488-conjugated goat anti rabbit IgG was used as the secondary antibody.

Application

Fig8: Flow cytometric analysis of SH-SY-5Y cells with Niemann Pick C1 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black). Alexa Fluor 488-conjugated goat anti rabbit IgG was used as the secondary antibody.

Application

Fig8: Flow cytometric analysis of SH-SY-5Y cells with Niemann Pick C1 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black). Alexa Fluor 488-conjugated goat anti rabbit IgG was used as the secondary antibody.

Application

Fig8: Flow cytometric analysis of SH-SY-5Y cells with Niemann Pick C1 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black). Alexa Fluor 488-conjugated goat anti rabbit IgG was used as the secondary antibody.

Application

Fig8: Flow cytometric analysis of SH-SY-5Y cells with Niemann Pick C1 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black). Alexa Fluor 488-conjugated goat anti rabbit IgG was used as the secondary antibody.
Positive Control SiHa, A549, HepG2, PC-3M, Rat kidney tissue, human kidney tissue, mouse testis tissue, SH-SY-5Y.
Application Notes WB:1:500-1:1,000
ICC/IF:1:50-1:200
IHC:1:50-1:200
FC:1:50-1:100
Additional Information
Form Liquid
Storage Instructions Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles.
Storage Buffer 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
validation
question title
question detaiil
Verification code