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AB153425

Recombinant Human Twinkle protein (GST tag N-Terminus)

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Recombinant Human Twinkle protein (GST tag N-Terminus) is a Human Full Length protein, in the 1 to 506 aa range, expressed in Wheat germ, suitable for ELISA, WB.

View Alternative Names

C10orf2, PEO1, TWNK, Twinkle mtDNA helicase, Progressive external ophthalmoplegia 1 protein, T7 gp4-like protein with intramitochondrial nucleoid localization, T7-like mitochondrial DNA helicase

1 Images
SDS-PAGE - Recombinant Human Twinkle protein (GST tag N-Terminus) (AB153425)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human Twinkle protein (GST tag N-Terminus) (AB153425)

ab153425 on a 12.5% SDS-PAGE stained with Coomassie Blue.

Key facts

Expression system

Wheat germ

Tags

GST tag N-Terminus

Applications

ELISA, WB

applications

Biologically active

No

Accession

Q96RR1

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 0.79% Tris HCl, 0.31% Glutathione

storage-buffer

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Complementary Products

Primary Antibodies

AB129075

Anti-MFF antibody [EPR7360]

RabMAb|Recombinant

Western blot - Anti-MFF antibody [EPR7360] (AB129075)

  • 0
  • 1
Primary Antibodies

AB184247

Anti-DRP1 antibody [EPR19274]

20ul selling size|RabMAb|Recombinant

Western blot - Anti-DRP1 antibody [EPR19274] (AB184247)

  • 5
  • 5
Primary Antibodies

AB157457

Anti-OPA1 antibody [EPR11057(B)]

RabMAb|Recombinant

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-OPA1 antibody [EPR11057(B)] (AB157457)

  • 4
  • 5
Primary Antibodies

AB124773

Anti-Mitofusin 2 antibody [NIAR164]

KO Validated|RabMAb|Recombinant

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Mitofusin 2 antibody [NIAR164] (AB124773)

  • 4
  • 6
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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Sequence info

[{"sequence":"MFGLTKVTDDTLKRFSVRYLRPARSLVFPWFSPGGSGLRGLKLLEAKCQGDGVSYEETTIPRPSAYHNLFGLPLISRRDAEVVLTSRELDSLALNQSTGLPTLTLPRGTTCLPPALLPYLEQFRRIVFWLGDDLRSWEAAKLFARKLNPKRCFLVRPGDQQPRPLEALNGGFNLSRILRTALPAWHKSIVSFRQLREEVLGELSNVEQAAGLRWSRFPDLNRILKGHRKGELTVFTGPTGSGKTTFISEYALDLCSQGVNTLWGSFEISNVRLARVMLTQFAEGRLEDQLDKYDHWADRFEDLPLYFMTFHGQQSIRTVIDTMQHAVYVYDICHVIIDNLQFMMGHEQLSTDRIAAQDYIIGVFRKFATDNNCHVTLVIHPRKEDDDKELQTASIFGSAKASQEADNVLILQDRKLVTGPGKRYLQVSKNRFDGDVGVFPLEFNKNSLTFSIPPKNKARLKKIKDDTGPVAKKPSSGKKGATTQNSEICSGQAPTPDQPDTSKRSK","proteinLength":"Full Length","predictedMolecularWeight":null,"actualMolecularWeight":null,"aminoAcidEnd":506,"aminoAcidStart":1,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"Q96RR1","tags":[{"tag":"GST","terminus":"N-Terminus"}]}]
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Properties and storage information

Shipped at conditions
Dry Ice
Appropriate short-term storage conditions
-80°C
Appropriate long-term storage conditions
-80°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

Twinkle also known as the mitochondrial DNA helicase functions as an essential component in mitochondrial DNA replication. The Twinkle protein unwinds DNA a necessary step for replication. Twinkle has a molecular mass of approximately 80 kDa. Twinkle expresses notably in cells with high energy demands such as muscle cells and neurons indicating its importance in tissues with substantial mitochondrial activity.
Biological function summary

Twinkle facilitates efficient mitochondrial DNA replication by acting within a complex including POLG (the mitochondrial DNA polymerase) and DNA helicase. This action ensures accurate mitochondrial DNA synthesis. Twinkle helps maintain mitochondrial genome stability supporting normal mitochondrial function and energy production.

Pathways

Twinkle plays a fundamental role in the mitochondrial DNA replication pathway. This process is critical for maintaining the genetic material necessary for mitochondrial biogenesis and function. Twinkle acts alongside proteins such as POLG restoring and replicating mitochondrial DNA thereby sustaining cellular energy metabolism and mitochondrial integrity.

Twinkle mutations associate with several mitochondrial DNA depletion syndromes including progressive external ophthalmoplegia and adult-onset multisystem disorders. Dysregulation of Twinkle activity and resulting mitochondrial dysfunction connect to defective POLG activity leading to impaired energy metabolism and associated clinical symptoms. Understanding Twinkle’s role helps to elucidate the molecular basis of these conditions and guides potential therapeutic strategies.
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Specifications

Form

Liquid

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General info

Function

Isoform 1. Mitochondrial helicase involved in mtDNA replication and repair (PubMed : 12975372, PubMed : 15167897, PubMed : 17324440, PubMed : 18039713, PubMed : 18971204, PubMed : 25824949, PubMed : 26887820, PubMed : 27226550). Might have a role in mtDNA repair (PubMed : 27226550). Has DNA strand separation activity needed to form a processive replication fork for leading strand synthesis which is catalyzed by the formation of a replisome complex with POLG and mtSDB (PubMed : 12975372, PubMed : 15167897, PubMed : 18039713, PubMed : 22383523, PubMed : 26887820, PubMed : 27226550). Preferentially unwinds DNA substrates with pre-existing 5'-and 3'- single-stranded tails but is also active on a 5'- flap substrate (PubMed : 12975372, PubMed : 15167897, PubMed : 18039713, PubMed : 22383523, PubMed : 26887820, PubMed : 27226550). Can dissociate the invading strand of immobile or mobile D-loop DNA structures irrespective of the single strand polarity of the third strand (PubMed : 27226550). In addition to its DNA strand separation activity, also has DNA strand annealing, DNA strand-exchange and DNA branch migration activities (PubMed : 22383523, PubMed : 26887820, PubMed : 27226550).. Isoform 2. Lack DNA unwinding and ATP hydrolysis activities (PubMed : 18039713). Does not bind single-stranded or double-stranded DNA (PubMed : 18039713).

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Product protocols

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Target data

Isoform 1. Mitochondrial helicase involved in mtDNA replication and repair (PubMed : 12975372, PubMed : 15167897, PubMed : 17324440, PubMed : 18039713, PubMed : 18971204, PubMed : 25824949, PubMed : 26887820, PubMed : 27226550). Might have a role in mtDNA repair (PubMed : 27226550). Has DNA strand separation activity needed to form a processive replication fork for leading strand synthesis which is catalyzed by the formation of a replisome complex with POLG and mtSDB (PubMed : 12975372, PubMed : 15167897, PubMed : 18039713, PubMed : 22383523, PubMed : 26887820, PubMed : 27226550). Preferentially unwinds DNA substrates with pre-existing 5'-and 3'- single-stranded tails but is also active on a 5'- flap substrate (PubMed : 12975372, PubMed : 15167897, PubMed : 18039713, PubMed : 22383523, PubMed : 26887820, PubMed : 27226550). Can dissociate the invading strand of immobile or mobile D-loop DNA structures irrespective of the single strand polarity of the third strand (PubMed : 27226550). In addition to its DNA strand separation activity, also has DNA strand annealing, DNA strand-exchange and DNA branch migration activities (PubMed : 22383523, PubMed : 26887820, PubMed : 27226550).. Isoform 2. Lack DNA unwinding and ATP hydrolysis activities (PubMed : 18039713). Does not bind single-stranded or double-stranded DNA (PubMed : 18039713).
See full target information TWNK
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