Anti-Niemann Pick C1 antibody (ab177233)
Key features and details
- Goat polyclonal to Niemann Pick C1
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-Niemann Pick C1 antibody
See all Niemann Pick C1 primary antibodies -
Description
Goat polyclonal to Niemann Pick C1 -
Host species
Goat -
Tested applications
Suitable for: WBmore details -
Species reactivity
Reacts with: Human
Predicted to work with: Pig -
Immunogen
Synthetic peptide corresponding to Human Niemann Pick C1 aa 1040-1053 (internal sequence) (Cysteine residue).
Sequence:TYHTVLQTSADFID
Database link: NP_000262.2 -
Positive control
- HEK293 cell lysate.
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General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 99% Tris buffered saline, 0.5% BSA -
Concentration information loading...
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Purity
Immunogen affinity purified -
Purification notes
ab177233 was purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide. -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
- Metabolism
- Pathways and Processes
- Metabolic signaling pathways
- Lipid and lipoprotein metabolism
- Cholesterol Metabolism
- Metabolism
- Pathways and Processes
- Metabolic signaling pathways
- Lipid and lipoprotein metabolism
- Lipoprotein metabolism
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
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Related Products
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab177233 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application | Abreviews | Notes |
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WB |
Use a concentration of 0.5 - 1.5 µg/ml. Detects a band of approximately 170 kDa (predicted molecular weight: 142 kDa).
1 hour primary incubation is recommended for this product. |
Notes |
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WB
Use a concentration of 0.5 - 1.5 µg/ml. Detects a band of approximately 170 kDa (predicted molecular weight: 142 kDa). 1 hour primary incubation is recommended for this product. |
Target
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Function
Involved in the intracellular trafficking of 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. -
Involvement in disease
Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPDC1) [MIM:257220]. 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 C1 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. An allelic variant of Niemann-Pick disease type C1 is found in people with Nova Scotia ancestry. Patients with the Nova Scotian clinical variant are less severely affected. -
Sequence similarities
Belongs to the patched family.
Contains 1 SSD (sterol-sensing) domain. -
Domain
A cysteine-rich N-terminal domain and a C-terminal domain containing a di-leucine motif necessary for lysosomal targeting are critical for mobilization of cholesterol from lysosomes. -
Post-translational
modificationsGlycosylated. -
Cellular localization
Late endosome membrane. Lysosome membrane. - Information by UniProt
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Database links
- Entrez Gene: 4864 Human
- Entrez Gene: 397591 Pig
- Omim: 607623 Human
- SwissProt: O15118 Human
- SwissProt: P56941 Pig
- Unigene: 464779 Human
- Unigene: 715623 Human
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Alternative names
- Niemann Pick C1 protein precursor antibody
- Niemann Pick disease, type C1 antibody
- Niemann-Pick C1 protein antibody
see all
Images
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Anti-Niemann Pick C1 antibody (ab177233) at 0.5 µg/ml + HEK293 cell lysate (in RIPA buffer) at 35 µg
Developed using the ECL technique.
Predicted band size: 142 kDa
Observed band size: 170 kDa why is the actual band size different from the predicted?Primary incubation was 1 hour.
Datasheets and documents
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Datasheet download
References (0)
ab177233 has not yet been referenced specifically in any publications.