NB400-148

antibody from Novus Biologicals

Targeting: NPC1 SLC65A1

Western blot (Supportive data in Antibodypedia)

Immunocytochemistry (Data presented on provider website)

Immunoprecipitation (Data presented on provider website)

Immunohistochemistry (Supportive data in Antibodypedia)

Immunoelectron microscopy (Data presented on provider website)

Antibody Data

Product number

NB400-148

Provider

Novus Biologicals

Proper citation

Novus Cat#NB400-148, RRID:AB_10001101

Product name

Rabbit Polyclonal Niemann-Pick C1 Antibody

Antibody type

Polyclonal

Description

Immunogen affinity purified.

Reactivity

Human, Mouse, Rat, Hamster, Porcine, Simian

Host

Rabbit

Isotype

IgG

Vial size

0.1 ml

Concentration

1.0 mg/ml

Storage

Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.

References

Submitted references

Restoration of lysosomal function after damage is accompanied by recycling of lysosomal membrane proteins.

Eriksson I, Wäster P, Öllinger K
Cell death & disease 2020 May 14;11(5):370

---

Molecular Genetics of Niemann-Pick Type C Disease in Italy: An Update on 105 Patients and Description of 18 NPC1 Novel Variants.

Dardis A, Zampieri S, Gellera C, Carrozzo R, Cattarossi S, Peruzzo P, Dariol R, Sechi A, Deodato F, Caccia C, Verrigni D, Gasperini S, Fiumara A, Fecarotta S, Carecchio M, Filosto M, Santoro L, Borroni B, Bordugo A, Brancati F, Russo CV, Di Rocco M, Toscano A, Scarpa M, Bembi B
Journal of clinical medicine 2020 Mar 3;9(3)

---

Different Trafficking Phenotypes of Niemann-Pick C1 Gene Mutations Correlate with Various Alterations in Lipid Storage, Membrane Composition and Miglustat Amenability.

Brogden G, Shammas H, Walters F, Maalouf K, Das AM, Naim HY, Rizk S
International journal of molecular sciences 2020 Mar 19;21(6)

---

NPC1 enables cholesterol mobilization during long-term potentiation that can be restored in Niemann-Pick disease type C by CYP46A1 activation.

Mitroi DN, Pereyra-Gómez G, Soto-Huelin B, Senovilla F, Kobayashi T, Esteban JA, Ledesma MD
EMBO reports 2019 Nov 5;20(11):e48143

---

Lysosomal integral membrane protein-2 (LIMP-2/SCARB2) is involved in lysosomal cholesterol export.

Heybrock S, Kanerva K, Meng Y, Ing C, Liang A, Xiong ZJ, Weng X, Ah Kim Y, Collins R, Trimble W, Pomès R, Privé GG, Annaert W, Schwake M, Heeren J, Lüllmann-Rauch R, Grinstein S, Ikonen E, Saftig P, Neculai D
Nature communications 2019 Aug 6;10(1):3521

---

Reduction of TMEM97 increases NPC1 protein levels and restores cholesterol trafficking in Niemann-pick type C1 disease cells.

Ebrahimi-Fakhari D, Wahlster L, Bartz F, Werenbeck-Ueding J, Praggastis M, Zhang J, Joggerst-Thomalla B, Theiss S, Grimm D, Ory DS, Runz H
Human molecular genetics 2016 Aug 15;25(16):3588-3599

---

Stimulation of adenosine A2A receptors reduces intracellular cholesterol accumulation and rescues mitochondrial abnormalities in human neural cell models of Niemann-Pick C1.

Ferrante A, De Nuccio C, Pepponi R, Visentin S, Martire A, Bernardo A, Minghetti L, Popoli P
Neuropharmacology 2016 Apr;103:155-62

---

Glycosylation inhibition reduces cholesterol accumulation in NPC1 protein-deficient cells.

Li J, Deffieu MS, Lee PL, Saha P, Pfeffer SR
Proceedings of the National Academy of Sciences of the United States of America 2015 Dec 1;112(48):14876-81

---

Lysosomal network proteins as potential novel CSF biomarkers for Alzheimer's disease.

Armstrong A, Mattsson N, Appelqvist H, Janefjord C, Sandin L, Agholme L, Olsson B, Svensson S, Blennow K, Zetterberg H, Kågedal K
Neuromolecular medicine 2014 Mar;16(1):150-60

---

Pulmonary abnormalities in animal models due to Niemann-Pick type C1 (NPC1) or C2 (NPC2) disease.

Roszell BR, Tao JQ, Yu KJ, Gao L, Huang S, Ning Y, Feinstein SI, Vite CH, Bates SR
PloS one 2013;8(7):e67084

---

Efficacy of N-acetylcysteine in phenotypic suppression of mouse models of Niemann-Pick disease, type C1.

Fu R, Wassif CA, Yanjanin NM, Watkins-Chow DE, Baxter LL, Incao A, Liscum L, Sidhu R, Firnkes S, Graham M, Ory DS, Porter FD, Pavan WJ
Human molecular genetics 2013 Sep 1;22(17):3508-23

---

Adenovirus RIDα uncovers a novel pathway requiring ORP1L for lipid droplet formation independent of NPC1.

Cianciola NL, Greene DJ, Morton RE, Carlin CR
Molecular biology of the cell 2013 Nov;24(21):3309-25

---

LDL cholesterol recycles to the plasma membrane via a Rab8a-Myosin5b-actin-dependent membrane transport route.

Kanerva K, Uronen RL, Blom T, Li S, Bittman R, Lappalainen P, Peränen J, Raposo G, Ikonen E
Developmental cell 2013 Nov 11;27(3):249-62

---

Discovery of oxysterol-derived pharmacological chaperones for NPC1: implication for the existence of second sterol-binding site.

Ohgane K, Karaki F, Dodo K, Hashimoto Y
Chemistry & biology 2013 Mar 21;20(3):391-402

---

A regulatory role for microRNA 33* in controlling lipid metabolism gene expression.

Goedeke L, Vales-Lara FM, Fenstermaker M, Cirera-Salinas D, Chamorro-Jorganes A, Ramírez CM, Mattison JA, de Cabo R, Suárez Y, Fernández-Hernando C
Molecular and cellular biology 2013 Jun;33(11):2339-52

---

Treatment of Human Fibroblasts Carrying NPC1 Missense Mutations with MG132 Leads to an Improvement of Intracellular Cholesterol Trafficking.

Zampieri S, Bembi B, Rosso N, Filocamo M, Dardis A
JIMD reports 2012;2:59-69

---

Tracking sphingosine metabolism and transport in sphingolipidoses: NPC1 deficiency as a test case.

Blom T, Li Z, Bittman R, Somerharju P, Ikonen E
Traffic (Copenhagen, Denmark) 2012 Sep;13(9):1234-43

---

An essential role of Hrs/Vps27 in endosomal cholesterol trafficking.

Du X, Kazim AS, Brown AJ, Yang H
Cell reports 2012 Jan 26;1(1):29-35

---

Loss of Niemann Pick type C proteins 1 and 2 greatly enhances HIV infectivity and is associated with accumulation of HIV Gag and cholesterol in late endosomes/lysosomes.

Coleman EM, Walker TN, Hildreth JE
Virology journal 2012 Jan 24;9:31

---

A novel role for α-tocopherol transfer protein (α-TTP) in protecting against chloroquine toxicity.

Shichiri M, Kono N, Shimanaka Y, Tanito M, Rotzoll DE, Yoshida Y, Hagihara Y, Tamai H, Arai H
The Journal of biological chemistry 2012 Jan 20;287(4):2926-34

---

Imaging the impact of Nox4 in cycling hypoxia-mediated U87 glioblastoma invasion and infiltration.

Hsieh CH, Chang HT, Shen WC, Shyu WC, Liu RS
Molecular imaging and biology 2012 Aug;14(4):489-99

---

Subversion of NPC1 pathway of cholesterol transport by Anaplasma phagocytophilum.

Xiong Q, Rikihisa Y
Cellular microbiology 2012 Apr;14(4):560-76

---

Amiodarone impairs trafficking through late endosomes inducing a Niemann-Pick C-like phenotype.

Piccoli E, Nadai M, Caretta CM, Bergonzini V, Del Vecchio C, Ha HR, Bigler L, Dal Zoppo D, Faggin E, Pettenazzo A, Orlando R, Salata C, Calistri A, Palù G, Baritussio A
Biochemical pharmacology 2011 Nov 1;82(9):1234-49

---

Anatomically defined neuron-based rescue of neurodegenerative Niemann-Pick type C disorder.

Lopez ME, Klein AD, Dimbil UJ, Scott MP
The Journal of neuroscience : the official journal of the Society for Neuroscience 2011 Mar 23;31(12):4367-78

---

Dietary cholesterol induces trafficking of intestinal Niemann-Pick Type C1 Like 1 from the brush border to endosomes.

Skov M, Tønnesen CK, Hansen GH, Danielsen EM
American journal of physiology. Gastrointestinal and liver physiology 2011 Jan;300(1):G33-40

---

Cancer genetics-guided discovery of serum biomarker signatures for diagnosis and prognosis of prostate cancer.

Cima I, Schiess R, Wild P, Kaelin M, Schüffler P, Lange V, Picotti P, Ossola R, Templeton A, Schubert O, Fuchs T, Leippold T, Wyler S, Zehetner J, Jochum W, Buhmann J, Cerny T, Moch H, Gillessen S, Aebersold R, Krek W
Proceedings of the National Academy of Sciences of the United States of America 2011 Feb 22;108(8):3342-7

---

MLN64 mediates egress of cholesterol from endosomes to mitochondria in the absence of functional Niemann-Pick Type C1 protein.

Charman M, Kennedy BE, Osborne N, Karten B
Journal of lipid research 2010 May;51(5):1023-34

---

FTY720 stimulates 27-hydroxycholesterol production and confers atheroprotective effects in human primary macrophages.

Blom T, Bäck N, Mutka AL, Bittman R, Li Z, de Lera A, Kovanen PT, Diczfalusy U, Ikonen E
Circulation research 2010 Mar 5;106(4):720-9

---

Cyclodextrin overcomes deficient lysosome-to-endoplasmic reticulum transport of cholesterol in Niemann-Pick type C cells.

Abi-Mosleh L, Infante RE, Radhakrishnan A, Goldstein JL, Brown MS
Proceedings of the National Academy of Sciences of the United States of America 2009 Nov 17;106(46):19316-21

---

NPC1 repression contributes to lipid accumulation in human macrophages exposed to environmental aryl hydrocarbons.

Podechard N, Le Ferrec E, Rebillard A, Fardel O, Lecureur V
Cardiovascular research 2009 May 1;82(2):361-70

---

Transport of maternal cholesterol to the fetus is affected by maternal plasma cholesterol concentrations in the golden Syrian hamster.

Burke KT, Colvin PL, Myatt L, Graf GA, Schroeder F, Woollett LA
Journal of lipid research 2009 Jun;50(6):1146-55

---

Cholesterol sensor ORP1L contacts the ER protein VAP to control Rab7-RILP-p150 Glued and late endosome positioning.

Rocha N, Kuijl C, van der Kant R, Janssen L, Houben D, Janssen H, Zwart W, Neefjes J
The Journal of cell biology 2009 Jun 29;185(7):1209-25

---

Identification of cholesterol-regulating genes by targeted RNAi screening.

Bartz F, Kern L, Erz D, Zhu M, Gilbert D, Meinhof T, Wirkner U, Erfle H, Muckenthaler M, Pepperkok R, Runz H
Cell metabolism 2009 Jul;10(1):63-75

---

Niemann-Pick type C1 I1061T mutant encodes a functional protein that is selected for endoplasmic reticulum-associated degradation due to protein misfolding.

Gelsthorpe ME, Baumann N, Millard E, Gale SE, Langmade SJ, Schaffer JE, Ory DS
The Journal of biological chemistry 2008 Mar 28;283(13):8229-36

---

Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein.

Infante RE, Abi-Mosleh L, Radhakrishnan A, Dale JD, Brown MS, Goldstein JL
The Journal of biological chemistry 2008 Jan 11;283(2):1052-63

---

Cholesterol overload promotes morphogenesis of a Niemann-Pick C (NPC)-like compartment independent of inhibition of NPC1 or HE1/NPC2 function.

Frolov A, Srivastava K, Daphna-Iken D, Traub LM, Schaffer JE, Ory DS
The Journal of biological chemistry 2001 Dec 7;276(49):46414-21

---

Niemann-pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis.

Millard EE, Srivastava K, Traub LM, Schaffer JE, Ory DS
The Journal of biological chemistry 2000 Dec 8;275(49):38445-51

Western blot

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

Western Blot: Niemann-Pick C1 Antibody [NB400-148] - Detection of human NPC1 in 20 ug of human fibroblast cell lysate.

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

Western Blot: Niemann-Pick C1 Antibody [NB400-148] - HeLa lysate, BHK lysate, A431 lysate tested at 1:1000. Image from verified customer review.

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

Western Blot: Niemann-Pick C1 Antibody [NB400-148] - NPC proteins in mouse lungs. Western blot of wild type (W) littermates, NPC1 (Mut) or NPC2 (Mut) mutant mouse lungs using anti-NPC1 or -NPC2 antibody. beta-actin used as a loading control. 30 ug protein/lane. Image collected and cropped by CiteAb from the following publication (http://dx.plos.org/10.1371/journal.pone.0067084), licensed under a CC-BY licence.

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

Western Blot: Niemann-Pick C1 Antibody [NB400-148] - Protein expression analysis of normal and NPC-deficient cells after HIV-1 infection. Cells were uninfected or infected with VSVG-HIV-1 and harvested 96 h post-infection. NPC2, NPC1, and beta-actin protein expression was detected via Western blotting in uninfected and infected cells. Gag expression was also detected in the infected cells. Image collected and cropped by CiteAb from the following publication (http://virologyj.biomedcentral.com/articles/10.1186/1743-422X-9-31), licensed under a CC-BY licence.

Immunohistochemistry

Supportive validation

Submitted by

Novus Biologicals (provider)

Main image

Experimental details

Immunohistochemistry-Paraffin: Niemann-Pick C1 Antibody [NB400-148] - Staining of human brain, cortex, neurons and astrocytes.