Recombinant Human Mitochondrial thiamine pyrophosphate carrier (SLC25A19), partial

Code CSB-YP864025HU1
MSDS
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Source Yeast
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Code CSB-EP864025HU1
MSDS
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Source E.coli
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Code CSB-EP864025HU1-B
MSDS
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Source E.coli
Conjugate Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
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Code CSB-BP864025HU1
MSDS
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Source Baculovirus
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Code CSB-MP864025HU1
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
SLC25A19
Uniprot No.
Alternative Names
SLC25A19; DNC; MUP1; Mitochondrial thiamine pyrophosphate carrier; Mitochondrial uncoupling protein 1; Solute carrier family 25 member 19
Species
Homo sapiens (Human)
Protein Length
Partial
Tag Info
Tag type will be determined during the manufacturing process.
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Form
Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet
Please contact us to get it.

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Target Background

Function
Mitochondrial transporter mediating uptake of thiamine pyrophosphate (ThPP) into mitochondria.
Gene References into Functions
  1. Pancreatic acinar cells Mitochondrial Thiamin Pyrophosphate uptake process is adaptively regulated by the prevailing thiamin level and this regulation is exerted at the level of transcription of the SLC25A19 gene and involves transcription factor binding affinity/epigenetic mechanisms. PMID: 28729247
  2. Among 6 residues predicted by the docking model ( Thr(29), Arg(30), Ile(33), Ser(34), Asp(37) and Phe(298)), only Ile(33), Ser(34) and Asp(37) were critical for function. Ser(34) did not affect translation/stability. Ile(33) and Asp(37) mutants decreased this parameter andwere expressed less in mitochondria. A polar residue was needed at position 34. His(137) and Lys(291) are needed for delivery to mitochondria. PMID: 27188525
  3. Chronic alcohol exposure negatively impacts pancreatic mitochondrial thiamin pyrophosphate transport, and this effect is exerted, at least in part, at the level of Slc25a19 transcription and appears to involve an epigenetic mechanism. PMID: 26316591
  4. These findings demonstrate that the genes involved in dictating thiamine homeostasis, such as SLC19A2, SLC25A19 and TPK-1, were significantly up-regulated in clinical tissues and breast cancer cell lines. PMID: 23642734
  5. Characterization of the SLC25A19 promoter and demonstration of an essential role for NF-Y in its basal activity. PMID: 23872534
  6. mutant protein lacks the normal transport activity, implying that failed deoxynucleotide transport across the inner mitochondrial membrane causes Amiah microcephaly (MCPHA) PMID: 12185364
  7. mitochondria of Slc25a19(-/-) and Amish lethal microcephaly cells have undetectable and markedly reduced thiamine pyrophosphate content, respectively PMID: 17035501
  8. We review the evidence that the function of the SLC25A19 gene product, previously identified as the mitochondrial deoxyribonucleotide carrier (DNC), is actually the transport of thiamine pyrophosphate.[review] PMID: 18280798
  9. a pathogenic missense mutation in the SLC25A19 gene was identifiedin 4 patients who suffered from recurrent episodes of flaccid paralysis and encephalopathy associated with bilateral striatal necrosis and chronic progressive polyneuropathy PMID: 19798730

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Involvement in disease
Microcephaly, Amish type (MCPHA); Thiamine metabolism dysfunction syndrome 4, bilateral striatal degeneration and progressive polyneuropathy type (THMD4)
Subcellular Location
Mitochondrion inner membrane; Multi-pass membrane protein.
Protein Families
Mitochondrial carrier (TC 2.A.29) family
Tissue Specificity
Expressed in all tissues examined except for placenta. Highest levels in colon, kidney, lung, testis, spleen, and brain.
Database Links

HGNC: 14409

OMIM: 606521

KEGG: hsa:60386

STRING: 9606.ENSP00000319574

UniGene: Hs.514470

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