Recombinant Human Sterol carrier protein 2 (ab117049)
- Datasheet
- References
- Protocols
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 -
Additional notes
This protein is best used within three months from the date of receipt.
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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.
Datasheets and documents
References
ab117049 has not yet been referenced specifically in any publications.
