TWNK Antibody - middle region : Biotin (ARP36482_P050-Biotin)

Product Specifications

Gene Name

Twinkle mtDNA helicase

Gene Aliases

PEO, PEO1, SCA8, ATXN8, IOSCA, PEOA3, SANDO, TWINL, MTDPS7, PRLTS5, C10orf2

Gene ID

56652

Swiss Prot

Q96RR1

Accession Number

NP_068602

Host

Rabbit

Reactivity

Human, Mouse, Rat, Cow, Guinea Pig, Horse

Immunogen

The immunogen is a synthetic peptide directed towards the middle region of human PEO1

Target

This gene encodes a hexameric DNA helicase which unwinds short stretches of double-stranded DNA in the 5' to 3' direction and, along with mitochondrial single-stranded DNA binding protein and mtDNA polymerase gamma, is thought to play a key role in mtDNA replication. The protein localizes to the mitochondrial matrix and mitochondrial nucleoids. Mutations in this gene cause infantile onset spinocerebellar ataxia (IOSCA) and progressive external ophthalmoplegia (PEO) and are also associated with several mitochondrial depletion syndromes. Alternative splicing results in multiple transcript variants encoding distinct isoforms.

Partner Proteins

ZBTB1; BMI1; SMAD9; ICT1; SQSTM1; AKTIP

Clonality

Polyclonal

Conjugation

Biotin

Type

Polyclonal Antibody

Applications

Purification

Affinity Purified

Concentration

0.5 mg/ml

Homology

Cow: 86%; Guinea Pig: 79%; Horse: 79%; Human: 100%; Mouse: 86%; Rat: 86%

Format

Liquid. Purified antibody supplied in 1x PBS buffer.

Reconstitution

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.