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SLC25A19, Polyclonal Antibody

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產(chǎn)品名稱: SLC25A19, Polyclonal Antibody
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SLC25A19, Polyclonal Antibody


SLC25A19, Polyclonal Antibody  的詳細(xì)介紹
Product Name

SLC25A19, Polyclonal Antibody

Full Product Name

Rabbit Polyclonal to Human SLC25A19

Product Synonym Names
Anti-SLC25A19 Antibody (aa244-274) IHC-plus; SLC25A19; DNC; THMD4; MCPHA; TPC; Microcephaly; Amish; THMD3; Human SLC25A19
Product Gene Name

anti-SLC25A19 antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
phenotype 613710
3D Structure
ModBase 3D Structure for Q9HC21
Clonality
Polyclonal
Host
Rabbit
Species Reactivity
Human, Mouse
Specificity
Human SLC25A19
Purity/Purification
Immunoaffinity Purified
Form/Format
PBS, 0.09% sodium azide
Target Species
Human
Immunogen Description
KLH-conjugated synthetic peptide from C-terminal region of human SLC25A19.
Immunogen Type
Synthetic peptide
Immunogen
SLC25A19 antibody was raised against kLH-conjugated synthetic peptide from C-terminal region of human SLC25A19.
Antigen Modification
aa244-274
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.
Other Notes
Small volumes of anti-SLC25A19 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Categories/Family for anti-SLC25A19 antibody
Subfamily:-Mitochondrial-carrier">Family: Transporter
Subfamily: Mitochondrial carrier
Applications Tested/Suitable for anti-SLC25A19 antibody
Immunohistochemistry (IHC - Paraffin), Western Blot (WB)
Application Notes for anti-SLC25A19 antibody
IHC-P (1:100), WB (1:1000)

Immunohistochemistry (IHC) of anti-SLC25A19 antibody
Anti-SLC25A19 antibody IHC staining of human small intestine. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody dilution 1:100.
anti-SLC25A19 antibody Immunohistochemistry (IHC) (IHC) image
Western Blot (WB) of anti-SLC25A19 antibody
Western blot of SLC25A19 Antibody (C-term) in mouse cerebellum,heart tissue lysates (35 ug/lane).SLC25A19 (arrow) was detected using the purified Pab.
anti-SLC25A19 antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for SLC25A19. It may not necessarily be applicable to this product.
NCBI GI #
186928856
NCBI GeneID
60386
NCBI Accession #
NP_068380.3 [Other Products]
NCBI GenBank Nucleotide #
NM_021734.4 [Other Products]
UniProt Primary Accession #
Q9HC21 [Other Products]
UniProt Secondary Accession #
Q6V9R7; E9PF74[Other Products]
UniProt Related Accession #
Q9HC21[Other Products]
Molecular Weight
29,217 Da
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NCBI Official Full Name
mitochondrial thiamine pyrophosphate carrier
NCBI Official Synonym Full Names
solute carrier family 25 (mitochondrial thiamine pyrophosphate carrier), member 19
NCBI Official Symbol
SLC25A19??[Similar Products]
NCBI Official Synonym Symbols
DNC; TPC; MUP1; MCPHA; THMD3; THMD4
??[Similar Products]
NCBI Protein Information
mitochondrial thiamine pyrophosphate carrier; microcephaly, Amish; mitochondrial uncoupling protein 1; solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19
UniProt Protein Name
Mitochondrial thiamine pyrophosphate carrier
UniProt Synonym Protein Names
Mitochondrial uncoupling protein 1; Solute carrier family 25 member 19
Protein Family
Mitochondrial thiamine pyrophosphate carrier
UniProt Gene Name
SLC25A19??[Similar Products]
UniProt Synonym Gene Names
DNC; MUP1??[Similar Products]
UniProt Entry Name
TPC_HUMAN
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NCBI Summary for SLC25A19
This gene encodes a mitochondrial protein that is a member of the solute carrier family. Although this protein was initially thought to be the mitochondrial deoxynucleotide carrier involved in the uptake of deoxynucleotides into the matrix of the mitochondria, further studies have demonstrated that this protein instead functions as the mitochondrial thiamine pyrophosphate carrier, which transports thiamine pyrophosphates into mitochondria. Mutations in this gene cause microcephaly, Amish type, a metabolic disease that results in severe congenital microcephaly, severe 2-ketoglutaric aciduria, and death within the first year. Multiple alternatively spliced variants, encoding the same protein, have been identified for this gene. [provided by RefSeq, Jul 2008]
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UniProt Comments for SLC25A19
SLC25A19: Mitochondrial transporter mediating uptake of thiamine pyrophosphate (ThPP) into mitochondria. Defects in SLC25A19 are the cause of microcephaly Amish type (MCPHA); also known as Amish lethal microcephaly. MCPHA is an autosomal recessive metabolic disorder characterized by severe congenital microcephaly, severe 2- ketoglutaric aciduria and death within the first year. Defects in SLC25A19 are the cause of striatal necrosis bilateral and progressive polyneuropathy (SNBPP). A disease characterized by recurrent episodes of flaccid paralysis and encephalopathy associated with bilateral striatal necrosis and chronic progressive polyneuropathy. Belongs to the mitochondrial carrier family.

Protein type: Transporter, SLC family; Membrane protein, multi-pass; Transporter; Mitochondrial; Membrane protein, integral

Chromosomal Location of Human Ortholog: 17q25.3

Cellular Component: mitochondrial inner membrane; integral to membrane; nucleus

Molecular Function: deoxynucleotide transmembrane transporter activity

Biological Process: deoxynucleotide transport; transmembrane transport

Disease: Thiamine Metabolism Dysfunction Syndrome 4 (bilateral Striatal Degeneration And Progressive Polyneuropathy Type); Microcephaly, Amish Type
Research Articles on SLC25A19
1. 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.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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