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Key Documents

SAB1401382

Sigma-Aldrich

Anti-TFAM antibody produced in mouse

purified immunoglobulin, buffered aqueous solution

Synonym(s):

MtTF1, TCF6, TCF6L1, TCF6L2, TCF6L3, mtTFA

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

mouse

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

species reactivity

human

technique(s)

direct immunofluorescence: suitable
immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
western blot: 1 μg/mL

NCBI accession no.

NM_003201.1

UniProt accession no.

Q00059

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... TFAM(7019)

Related Categories

General description

This gene encodes a mitochondrial transcription factor that is a key activator of mitochondrial transcription as well as a participant in mitochondrial genome replication. Studies in mice have demonstrated that this gene product is required to regulate the mitochondrial genome copy number and is essential for embryonic development. A mouse model for Kearns-Sayre syndrome was produced when expression of this gene was eliminated by targeted disruption in heart and muscle cells. (provided by RefSeq)
Transcription factor A, mitochondrial (TFAM) is encoded by the gene mapped to human chromosome 10q21.1. The gene codes for a DNA binding protein characterized with HMG (high mobility group)-box domains.

Immunogen

TFAM (NP_003192.1, 1 a.a. ~ 246 a.a) full-length human protein.

Sequence
MAFLRSMWGVLSALGRSGAELCTGCGSRLRSPFSFVYLPRWFSSVLASCPKKPVSSYLRFSKEQLPIFKAQNPDAKTTELIRRIAQRWRELPDSKKKIYQDAYRAEWQVYKEEISRFKEQLTPSQIMSLEKEIMDKHLKRKAMTKKKELTLLGKPKRPRSAYNVYVAERFQEAKGDSPQEKLKTVKENWKNLSDSEKELYIQHAKEDETRYHNEMKSWEEQMIEVGRKDLLRRTIKKQRKYGAEEC

Biochem/physiol Actions

Transcription factor A, mitochondrial (TFAM) plays a vital role in transcription and replication of mammalian mitochondrial DNA (mtDNA). In addition, it is also implicated in mtDNA packaging. Polymorphism in the gene increases the risk of susceptibility to Alzheimer disease (AD). Additionally, mutation in the gene is associated with the development of mtDNA depletion syndrome.

Physical form

Solution in phosphate buffered saline, pH 7.4

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Storage Class Code

10 - Combustible liquids

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Mutations in TFAM, encoding mitochondrial transcription factor A, cause neonatal liver failure associated with mtDNA depletion.
Stiles A R, et al.
Molecular genetics and metabolism reports, 119(1), 91-99 (2016)
Possible association of mitochondrial transcription factor A (TFAM) genotype with sporadic Alzheimer disease
Gunther C, et al.
Neuroscience Letters, 369(3), 219-223 (2004)
PGC-1alpha downstream transcription factors NRF-1 and TFAM are genetic modifiers of Huntington disease
Taherzadeh-Fard E, et al.
Mol. Neurodegener., 6(1), 32-32 (2011)
Tfam, a mitochondrial transcription and packaging factor, imposes a U-turn on mitochondrial DNA
Ngo H B, et al.
Nature Structural and Molecular Biology, 18(11), 1290-1290 (2011)
Daniel A Pulliam et al.
The Biochemical journal, 462(2), 359-371 (2014-06-10)
Mutations in SURF1 (surfeit locus protein 1) COX (cytochrome c oxidase) assembly protein are associated with Leigh's syndrome, a human mitochondrial disorder that manifests as severe mitochondrial phenotypes and early lethality. In contrast, mice lacking the SURF1 protein (Surf1-/-) are
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