Rabbit Anti-NPC2 Recombinant Antibody (CBWJN-1495) (CBMAB-N3109-WJ)

Name: Rabbit Anti-NPC2 Recombinant Antibody (CBWJN-1495)
SKU: CBMAB-N3109-WJ
Rating: 5 (1 reviews)

See all NPC2 antibodies

Compare Online Inquiry

Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Rabbit

Clone

CBWJN-1495

Application

WB, ELISA, IP

Specificity

Human

Antibody Isotype

IgG

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

NPC Intracellular Cholesterol Transporter 2

Introduction

This gene encodes a protein containing a lipid recognition domain. The encoded protein may function in regulating the transport of cholesterol through the late endosomal/lysosomal system. Mutations in this gene have been associated with Niemann-Pick disease, type C2 and frontal lobe atrophy. [provided by RefSeq, Jul 2008]

Entrez Gene ID

10577

UniProt ID

P61916

Alternative Names

NPC Intracellular Cholesterol Transporter 2; Niemann-Pick Disease Type C2 Protein; Human Epididymis-Specific Protein 1; Epididymal Protein 1; HE1; Tissue-Specific Secretory Protein; Epididymal Secretory Protein E1; Niemann-Pick Disease, Type C2; EDDM1;

Function

Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the lysosomal compartment (PubMed:17018531, PubMed:11125141, PubMed:18772377, PubMed:29580834, PubMed:15937921).
Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol-binding pocket in the N-terminal domain of NPC1 (PubMed:17018531, PubMed:18772377, PubMed:27238017).
May bind and mobilize cholesterol that is associated with membranes (PubMed:18823126).
NPC2 binds cholesterol with a 1:1 stoichiometry (PubMed:17018531).
Can bind a variety of sterols, including lathosterol, desmosterol and the plant sterols stigmasterol and beta-sitosterol (PubMed:17018531).
The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport (By similarity).

Biological Process

Cholesterol effluxManual Assertion Based On ExperimentIDA:BHF-UCL
Cholesterol homeostasisManual Assertion Based On ExperimentIDA:UniProtKB
Cholesterol metabolic processIEA:UniProtKB-KW
Cholesterol transportManual Assertion Based On ExperimentIDA:UniProtKB
Glycolipid transportManual Assertion Based On ExperimentTAS:HGNC-UCL
Intracellular cholesterol transportManual Assertion Based On ExperimentIDA:UniProtKB
Intracellular sterol transportManual Assertion Based On ExperimentIDA:HGNC-UCL
Phospholipid transportManual Assertion Based On ExperimentTAS:HGNC-UCL
Regulation of isoprenoid metabolic processManual Assertion Based On ExperimentTAS:UniProtKB
Response to virusManual Assertion Based On ExperimentIEP:UniProtKB
Sterol transportManual Assertion Based On ExperimentIBA:GO_Central

Cellular Location

Secreted
Endoplasmic reticulum
Lysosome
Interaction with cell-surface M6PR mediates endocytosis and targeting to lysosomes.

Involvement in disease

Niemann-Pick disease C2 (NPC2):
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 C2 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.

Qian, H., Wu, X., Du, X., Yao, X., Zhao, X., Lee, J., ... & Yan, N. (2020). Structural basis of low-pH-dependent lysosomal cholesterol egress by NPC1 and NPC2. Cell, 182(1), 98-111.

Cologna, S. M., & Rosenhouse-Dantsker, A. (2019). Insights into the molecular mechanisms of cholesterol binding to the NPC1 and NPC2 proteins. Direct Mechanisms in Cholesterol Modulation of Protein Function, 139-160.

McCauliff, L. A., Langan, A., Li, R., Ilnytska, O., Bose, D., Waghalter, M., ... & Storch, J. (2019). Intracellular cholesterol trafficking is dependent upon NPC2 interaction with lysobisphosphatidic acid. Elife, 8, e50832.

Xu, Y., Zhang, Q., Tan, L., Xie, X., & Zhao, Y. (2019). The characteristics and biological significance of NPC2: Mutation and disease. Mutation Research/Reviews in Mutation Research, 782, 108284.

Ask a question We look forward to hearing from you.

0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Have you used Rabbit Anti-NPC2 Recombinant Antibody (CBWJN-1495)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon

$30 eGift Card
Submit a review

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Antibody Pairs

NPC2 Matched Antibody Pair (811) (CAT#: APMAB-811LY)

NPC2 Matched Antibody Pair (812) (CAT#: APMAB-812LY)

Related Products

Rabbit Anti-NPC2 Recombinant Antibody (CBWJN-1496) (CAT#: CBMAB-N3110-WJ)

Mouse Anti-NPC2 Recombinant Antibody (V2-177615) (CAT#: CBMAB-N3113-WJ)

Rabbit Anti-NPC2 Recombinant Antibody (BA0188) (CAT#: CBMAB-0535CQ)

NPC2 Matched Antibody Pair (811) (CAT#: APMAB-811LY)

Mouse Anti-NPC2 Recombinant Antibody (1F4) (CAT#: CBMAB-N3112-WJ)

NPC2 Matched Antibody Pair (812) (CAT#: APMAB-812LY)

Rabbit Anti-NPC2 Recombinant Antibody (BA0189) (CAT#: CBMAB-0536CQ)

Rabbit Anti-NPC2 Recombinant Antibody (CBWJN-1494) (CAT#: CBMAB-N3108-WJ)

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Request bulk or custom quote Second antibody and isotype control

Contact us

Tel: (USA)
(UK)
Fax:

Global Locations

Email:

Online Inquiry