Niemann Pick C1 Like 1 Antibody (YA9684)
(Synonyms: Niemann Pick C1 protein precursor, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC, NPC1, NPC1_HUMAN)
Based on 1 Customer Validation
Niemann Pick C1 Like 1 Antibody (YA9684) is a Rabbit-derived and non-conjugated IgG Recombinant,Monoclonal antibody, targeting to Niemann Pick C1 Like 1.
-
Host:
Rabbit
-
Isotype:
IgG
-
Application:
WB, ICC/IF, IF-Tissue, IHC-P, FC
-
Reactivity :
Human, Mouse, Rat
-
Formulation:
Supplied in TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
-
Conjugation:
Non-conjugated
To place orders, for customer services and technical support, please contact: MedChemExpress USA
Tel: 609-228-6898 E-mail: [email protected] [email protected]
Applications
| Application |
WB
WB: Western Blot
|
ICC/IF
ICC/IF: Immunocytochemistry/
Immunofluorescence |
IF-Tissue
IF-Tissue: Immunofluorescence-Tissue
|
IHC-P
IHC-P: Immunohistochemistry-Paraffin
|
FC
FC: Flow Cytometry
|
|---|---|---|---|---|---|
| Dilution Ratio | 1:500-2000 | 1:50-200 | 1:50-200 | 1:50-200 | 1:50-100 |
Product Details
Niemann Pick C1 Like 1 Antibody (YA9684) is a Rabbit-derived and non-conjugated IgG Recombinant,Monoclonal antibody, targeting to Niemann Pick C1 Like 1.
-
Host Rabbit
-
Clonality Recombinant,Monoclonal
-
Species ReactivityHuman, Mouse, Rat
-
Observed Molecular WeightObserved band size: 190 kDa
Note: Due to possible protein modifications or aggregation, the molecular weight should be confirmed by actual measurement, and the predicted value is for reference only.
-
Calculated Molecular Weight Predicted band size: 142 kDa
Recombinant protein of Human Niemann Pick C1 (aa 1160-1278).
Endogenous
affinity purified.
Non-conjugated
Unmodified
IgG
Product Properties
-
Appearance
Solution
-
Formulation
Supplied in TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
-
Concentration
Batch-dependent, Please check the COA for the concentration of each lot. Check Lot Concentration
-
Storage & Stability
Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.
-
Shipping
Shipping with blue ice.
Background
-
Function
Niemann Pick C1 Like 1 is an Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. 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. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket. Mediates cholesterol transfer from lysosomes to endoplasmic reticulum by tethering endosomal/lysosomal compartments and endoplasmic reticulum membrane contact sites where it interacts with sterol transport protein GRAMD1B. 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 (Probable). Inhibits cholesterol-mediated mTORC1 activation throught its interaction with SLC38A9[1][2][3][4][5][6][7][8][9][10][11].
-
Subcellular Localization
Late endosome membrane; Lysosome membrane
-
Isoforms & Post-Translational Modification
Niemann Pick C1 Like 1 has 2 isoforms, O15118-1: amino acid length is 1278, molecular weight is 142167 Da (predicted); O15118-2: amino acid length is 960, molecular weight is 107025 Da (predicted).N-glycosylated
-
Subunit
Interacts (via the second lumenal domain) with NPC2.
-
SwissProt ID
-
Synonyms
Niemann Pick C1 protein precursor, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC, NPC1, NPC1_HUMAN
Documentation
References
[1]. Davies JP, et al. Topological analysis of Niemann-Pick C1 protein reveals that the membrane orientation of the putative sterol-sensing domain is identical to those of 3-hydroxy-3-methylglutaryl-CoA reductase and sterol regulatory element binding protein cleavage-activating protein. J Biol Chem. 2000 Aug 11;275(32):24367-74. [Content Brief]
[2]. Blom TS, et al. Defective endocytic trafficking of NPC1 and NPC2 underlying infantile Niemann-Pick type C disease. Hum Mol Genet. 2003 Feb 1;12(3):257-72. [Content Brief]
[3]. Infante RE, et al. NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes. Proc Natl Acad Sci U S A. 2008 Oct 7;105(40):15287-92. [Content Brief]
[4]. Gong X, et al. Structural Insights into the Niemann-Pick C1 (NPC1)-Mediated Cholesterol Transfer and Ebola Infection. Cell. 2016 Jun 2;165(6):1467-1478. [Content Brief]
[5]. Carstea ED, et al. Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science. 1997 Jul 11;277(5323):228-31. [Content Brief]
[6]. Watari H, et al. Niemann-Pick C1 protein: obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization. Proc Natl Acad Sci U S A. 1999 Feb 2;96(3):805-10. [Content Brief]
[7]. Kwon HJ, et al. Structure of N-terminal domain of NPC1 reveals distinct subdomains for binding and transfer of cholesterol. Cell. 2009 Jun 26;137(7):1213-24. [Content Brief]
[8]. Ebrahimi-Fakhari D, et al. Reduction of TMEM97 increases NPC1 protein levels and restores cholesterol trafficking in Niemann-pick type C1 disease cells. Hum Mol Genet. 2016 Aug 15;25(16):3588-3599. [Content Brief]
[9]. Li X, et al. 3.3 Å structure of Niemann-Pick C1 protein reveals insights into the function of the C-terminal luminal domain in cholesterol transport. Proc Natl Acad Sci U S A. 2017 Aug 22;114(34):9116-9121. [Content Brief]
[10]. Höglinger D, et al. NPC1 regulates ER contacts with endocytic organelles to mediate cholesterol egress. Nat Commun. 2019 Sep 19;10(1):4276. [Content Brief]
[11]. Castellano BM, et al. Lysosomal cholesterol activates mTORC1 via an SLC38A9-Niemann-Pick C1 signaling complex. Science. 2017 Mar 24;355(6331):1306-1311. [Content Brief]
Powered by Bioz
