Recombinant Human Sterol carrier protein 2 (ab117049)
Key features and details
- Expression system: Wheat germ
- Suitable for: WB, ELISA, SDS-PAGE
Description
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Product name
Recombinant Human Sterol carrier protein 2 -
Expression system
Wheat germ -
Accession
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Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
MGFPEAASSFRTHQIEAVPTSSASDGFKANLVFKEIEKKLEEEGEQFVKK IGGIFAFKVKDGPGGKEATWVVDVKNGKGSVLPNSDKKADCTITMADSDF LALMTGKMNPQSAFFQGKLKITGNMGLAMKLQNLQLQPGNAKL -
Predicted molecular weight
42 kDa including tags -
Amino acids
1 to 143
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Specifications
Our Abpromise guarantee covers the use of ab117049 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
Western blot
ELISA
SDS-PAGE
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Form
Liquid -
Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00
Constituents: 0.3% Glutathione, 0.79% Tris HCl
General Info
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Alternative names
- DKFZp686C12188
- DKFZp686D11188
- NLTP
see all -
Function
Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis. -
Tissue specificity
Liver, fibroblasts, and placenta. -
Involvement in disease
Defects in SCP2 are a cause of leukoencephalopathy with dystonia and motor neuropathy (LDMN) [MIM:613724]; also known as sterol carrier protein 2 deficiency. LDMN is a syndrome characterized by leukoencephalopathy, dystonic head tremor, spasmodic torticollis and reduced tendon reflexes in lower extremities. Additional features include hyposmia, pathologic saccadic eye movements, a slight hypoacusis, accumulation of branched-chain pristanic acid in plasma, and the presence of abnormal bile alcohol glucuronides in urine. -
Sequence similarities
In the N-terminal section; belongs to the thiolase family.
Contains 1 SCP2 domain. -
Cellular localization
Mitochondrion; Cytoplasm. Mitochondrion. Cytoplasmic in the liver and also associated with mitochondria especially in steroidogenic tissues and Peroxisome. Interaction with PEX5 is essential for peroxisomal import. - Information by UniProt
Images
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SDS-PAGE - Recombinant Human Sterol carrier protein 2 (ab117049)12.5% SDS-PAGE showing ab117049 at approximately 41.84 kDa.
Stained with Coomassie Blue.
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
References (0)
ab117049 has not yet been referenced specifically in any publications.
