Antibody Name:	Anti-SLC25A19 Antibody
Antibody SKU:	CAB12373
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-80 of human SLC25A19 (NP_068380.3).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	HT-29

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-80 of human SLC25A19 (NP_068380.3).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	MVGY DPKP DGRN NTKF QVAV AGSV SGLV TRAL ISPF DVIK IRFQ LQHE RLSR SDPS AKYH GILQ ASRQ ILQE EGPT AFWK
Gene ID:	60386
Uniprot:	Q9HC21
Cellular Location:	Mitochondrion inner membrane, Multi-pass membrane protein
Calculated MW:	29kDa/35kDa
Observed MW:	36kDa

Synonyms:

SLC25A19, DNC, MCPHA, MUP1, THMD3, THMD4, TPC

Background:

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.

UniProt Protein Function:	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.
UniProt Protein Details:	Protein type:Membrane protein, integral; Transporter, SLC family; Transporter; Membrane protein, multi-pass; Mitochondrial Chromosomal Location of Human Ortholog: 17q25.3 Cellular Component: nucleus Molecular Function:deoxynucleotide transmembrane transporter activity; structural constituent of ribosome Biological Process: positive regulation of defense response to virus by host; translation Disease: Microcephaly, Amish Type; Thiamine Metabolism Dysfunction Syndrome 4 (bilateral Striatal Degeneration And Progressive Polyneuropathy Type)
NCBI Summary:	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]
UniProt Code:	Q9HC21
NCBI GenInfo Identifier:	20137652
NCBI Gene ID:	60386
NCBI Accession:	Q9HC21.1
UniProt Secondary Accession:	Q9HC21,Q6V9R7, E9PF74,
UniProt Related Accession:	Q9HC21
Molecular Weight:	29,217 Da
NCBI Full Name:	Mitochondrial thiamine pyrophosphate carrier
NCBI Synonym Full Names:	solute carrier family 25 member 19
NCBI Official Symbol:	SLC25A19
NCBI Official Synonym Symbols:	DNC; TPC; MUP1; MCPHA; THMD3; THMD4
NCBI Protein Information:	mitochondrial thiamine pyrophosphate carrier
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
UniProt Entry Name:	TPC_HUMAN

	Western blot analysis of extracts of HT-29 cells, using SLC25A19 antibody (CAB12373) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.
	Immunofluorescence analysis of NIH/3T3 cells using SLC25A19 antibody (CAB12373) at dilution of 1:100. Blue: DAPI for nuclear staining.