You are viewing an incomplete version of our website. Please click to reload the website as full version.

Niemann-Pick Disease, Type C1 Proteins (NPC1)

NPC1 encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. Additionally we are shipping NPC1 Antibodies (67) and NPC1 Kits (6) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
NPC1 18145 O35604
NPC1 4864 O15118
Rat NPC1 NPC1 266732  
How to order from antibodies-online
  • +1 877 302 8632
  • +1 888 205 9894 (toll-free)
  • Order online
  • orders@antibodies-online.com

Top NPC1 Proteins at antibodies-online.com

Showing 6 out of 10 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
Insect Cells Mouse rho-1D4 tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 0.25 mg Log in to see 69 to 74 Days
$9,050.32
Details
Insect Cells Human rho-1D4 tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 0.5 mg Log in to see 69 to 74 Days
$12,906.52
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
HOST_Human Cells Human His tag,DYKDDDDK Tag   100 μg Log in to see 16 Days
$437.80
Details

NPC1 Proteins by Origin and Source

Origin Expressed in Conjugate
Mouse (Murine) ,
,
Human , , ,
, , ,

More Proteins for Niemann-Pick Disease, Type C1 (NPC1) Interaction Partners

Zebrafish Niemann-Pick Disease, Type C1 (NPC1) interaction partners

  1. this is the first report, showing a role of NPC1 in platelet function and formation but further studies are needed to define how cholesterol storage interferes with these processes

  2. npc1 is required early for proper cell movement and cholesterol localization and later for cell survival

Rabbit Niemann-Pick Disease, Type C1 (NPC1) interaction partners

  1. Availability of assays to measure NPC1 binding to membrne proteins may further the understanding of ways in which oxysterols regulate intracellular lipid transport.

Mouse (Murine) Niemann-Pick Disease, Type C1 (NPC1) interaction partners

  1. Our data show that: i) HDAC2 (show HDAC2 Proteins) levels and activity are increased in NPC neuronal models and in Npc1(-/-) mice; ii) inhibition of c-Abl (show ABL1 Proteins) or c-Abl (show ABL1 Proteins) deficiency prevents the increase of HDAC2 (show HDAC2 Proteins) protein levels and activity in NPC neuronal models

  2. This study showed that deleting the Npc1 gene is accompanied by an increase in germ cell apoptosis and compensatory imbalances in the expression of cholesterol enzymatic and transporter factors.

  3. These results show that NPC1 is critical for ebolavirus replication and pathogenesis in animals.

  4. abnormal neuronal expression of matrix metalloproteinase-12 (show MMP12 Proteins) may contribute to axonal degeneration in Niemann-Pick type C disease

  5. There was a transient increase in biliary cholesterol concentration in Npc1(-/-) mice.

  6. data suggest that aberrant expression of Pcdhs is a pathological process accompanied by neurodegeneration in Npc1 mutant mice

  7. This study deministrated that lack of NPC1 in either neurons or glial cells did not affect the excitability of Purkinje cells, the formation of dendrites or their excitatory synaptic activity.

  8. Hearing loss is an early consequence of Npc1 gene deletion in the mouse model of Niemann-Pick disease, type C.

  9. These data show that cholesterol homeostasis through NPC1 plays a crucial role in maintaining insulin (show INS Proteins) action at multiple levels in adipocytes.

  10. Data suggest that axonal pathologies in Npc1 mutant spinal cord are strongly correlated with the increase of activated glial cells, which produce IL-1beta (show IL1B Proteins) and ApoE (show APOE Proteins), resulting in activation of p38-MAPK (show MAPK14 Proteins) signaling pathway and enhanced phosphorylated tau protein

Human Niemann-Pick Disease, Type C1 (NPC1) interaction partners

  1. Fibroblasts from Niemann-Pick type C (NPC) disease patients with low levels of NPC1 protein have high amounts of procathepsin D but reduced quantities of the mature protein, thus showing a diminished cathepsin D (show CTSD Proteins) activity.

  2. Results identified six novel mutations (PKHD1: p.Thr777Met, p.Tyr2260Cys; ABCB11 (show ABCB11 Proteins): p.Val1112Phe, c.611+1G > A, p.Gly628Trpfs*3 and NPC1: p.Glu391Lys) for the diagnostic of inherited infantile cholestatic disorders.

  3. NPC1 mutations are substantially enriched in unexplained early onset ataxia (show USP14 Proteins), making it high risk group for Niemann-Pick disease type C.

  4. these results clearly demonstrated that the over-expression of NPC1 with a defective function in an imatinib-resistant Ph+ acute lymphoblastic leukemia cell line

  5. Structure of glycosylated NPC1 luminal domain C reveals insights into NPC2 (show NPC2 Proteins) and Ebola virus interactions

  6. Study determined the crystal structure of the primed GP (GPcl) of Ebola virus bound to domain C of NPC1 (NPC1-C); NPC1-C utilizes two protruding loops to engage a hydrophobic cavity on head of GPcl. Upon enzymatic cleavage and NPC1-C binding, conformational change in the GPcl further affects the state of the internal fusion loop, triggering membrane fusion.

  7. Here, using live cell imaging, the authors obtained evidence that in contrast to the new model, ebolavirus enters cells through endolysosomes that contain both NPC1 and TPC2 (show TPCN2 Proteins).

  8. These experiments support a model in which NPC1 protein functions to transfer cholesterol past a lysosomal glycocalyx.

  9. An isobaric labeling-based quantitative analysis of proteome of NPC1(I1061T) primary fibroblasts when compared with wild-type cells identified 281 differentially expressed proteins based on stringent data analysis criteria, is reported.

  10. NPC1 gene sequencing revealed that he was a compound heterozygote for the p.S954L and p.N1156S mutations.

NPC1 Protein Profile

Protein Summary

This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.

Gene names and symbols associated with NPC1

  • Niemann-Pick disease, type C1 (NPC1)
  • Niemann-Pick disease, type C1 (npc1)
  • Niemann Pick type C1 (Npc1)
  • Niemann-Pick disease, type C1 (Npc1)
  • A430089E03Rik protein
  • C85354 protein
  • Cdig2 protein
  • D18Ertd139e protein
  • D18Ertd723e protein
  • im:7149020 protein
  • lcsd protein
  • nmf164 protein
  • NPC protein
  • spm protein
  • wu:fb53a12 protein
  • wu:fc29a12 protein

Protein level used designations for NPC1

Niemann-Pick C1 protein , Niemann-Pick type C1 disease protein , Nasopharyngeal carcinoma 1 , Niemann-Pick C1 , Niemann-Pick disease, type C1 , sphingomyelinosis , Niemann-Pick C disease protein

GENE ID SPECIES
403698 Canis lupus familiaris
455338 Pan troglodytes
493693 Felis catus
553330 Danio rerio
579887 Strongylocentrotus purpuratus
100008746 Oryctolagus cuniculus
18145 Mus musculus
4864 Homo sapiens
397591 Sus scrofa
421076 Gallus gallus
266732 Rattus norvegicus
100718604 Cavia porcellus
Selected quality suppliers for NPC1 Proteins (NPC1)
Did you look for something else?