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AB153425

Recombinant Human Twinkle protein (GST tag N-Terminus)

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.

別名を表示する

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

発現系

Wheat germ

タグ

GST tag N-Terminus

アプリケーション

WB, ELISA

applications

生物活性

No

アクセッション番号

Q96RR1

アニマルフリー

No

キャリアフリー

No

種

Human

バッファー組成

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

storage-buffer

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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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配列情報

[{"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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出荷温度及び保存条件

出荷温度
Dry Ice
短期保存温度
-80°C
長期保存温度
-80°C
分注に関する情報
Upon delivery aliquot
保管に関する情報
Avoid freeze / thaw cycle
False
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補足情報

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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製品の性状

製品の状態

Liquid

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一般的な情報

機能

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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製品プロトコール

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ターゲットの情報

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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組合せ製品

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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