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AB174417

Recombinant Human TIMM8A/DDP protein (His tag N-Terminus)

Recombinant Human TIMM8A/DDP protein (His tag N-Terminus)

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Recombinant Human TIMM8A/DDP protein (His tag N-Terminus) is a Human Full Length protein, in the 1 to 97 aa range, expressed in Escherichia coli, with >90%, suitable for SDS-PAGE, Mass Spec.

別名を表示する

DDP, DDP1, TIM8A, TIMM8A, Mitochondrial import inner membrane translocase subunit Tim8 A, Deafness dystonia protein 1, X-linked deafness dystonia protein

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SDS-PAGE - Recombinant Human TIMM8A/DDP protein (His tag N-Terminus) (AB174417)
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Supplier Data

SDS-PAGE - Recombinant Human TIMM8A/DDP protein (His tag N-Terminus) (AB174417)

15% SDS-PAGE analysis of ab174417 (3 μg)

Key facts

精製度

>90% SDS-PAGE

発現系

Escherichia coli

タグ

His tag N-Terminus

アプリケーション

Mass Spec, SDS-PAGE

applications

生物活性

No

アクセッション番号

O60220

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: 30% Glycerol (glycerin, glycerine), 0.88% Sodium chloride, 0.32% Tris-HCl buffer, 0.02% (R*,R*)-1,4-Dimercaptobutan-2,3-diol

storage-buffer

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "Mass Spec": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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製品の詳細

This product was previously labelled as TIMM8A
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配列情報

[{"sequence":"MDSSSSSSAAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD","proteinLength":"Full Length","predictedMolecularWeight":"13.4 kDa","actualMolecularWeight":null,"aminoAcidEnd":97,"aminoAcidStart":1,"nature":"Recombinant","expressionSystem":"Escherichia coli","accessionNumber":"O60220","tags":[{"tag":"His","terminus":"N-Terminus"}]}]
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出荷温度及び保存条件

出荷温度
Blue Ice
短期保存期間
1-2 weeks
短期保存温度
+4°C
長期保存温度
-20°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.

TIMM8A also known as DDP is a protein involved in mitochondrial import machinery important for transporting specific precursor proteins across the intermembrane space to the inner membrane. It has a molecular mass of about 10 kilodaltons. TIMM8A is predominantly expressed in mitochondria facilitating protein translocation through its interaction with carrier proteins. The protein is part of the TIM8-TIM13 complex important for delivering substrates to the TIM23 complex in the inner mitochondrial membrane ensuring the proper functioning of mitochondrial processes.
Biological function summary

In the context of mitochondria-dependent functions TIMM8A acts as a chaperone. It is a component of the TIM22 translocase complex that plays a critical role in inserting metabolite carrier proteins into the inner mitochondrial membrane. This activity is essential for maintaining mitochondrial integrity and ensuring energy production processes occur efficiently. TIMM8A partners with TIMM13 to form a hetero-oligomeric complex highlighting its integral role in translocating necessary proteins.

Pathways

Especially those related to cellular respiration and energy metabolism TIMM8A integrates into the mitochondrial import pathway. Its function relates closely to the TIM22 pathway which is responsible for the translocation and assembly of inner membrane carriers. TIMM8A cooperates with proteins such as TIMM9 and TIMM13 forming a complex that stabilizes the import intermediates. This cooperation ensures that imported proteins navigate correctly to their functional sites within mitochondria.

Proteins similar to TIMM8A are associated with Mohr-Tranebjaerg syndrome a disorder characterized by auditory and motor disturbances. Mutations affecting TIMM8A lead to dysfunction in the import machinery contributing to the pathophysiology of the syndrome. Furthermore its association with deafness-dystonia syndrome highlights TIMM8A's involvement in neurodegenerative conditions. These connections suggest potential interactions with related proteins like OPA1 known to impact mitochondrial dynamics and integrity further implicating TIMM8A in maintaining neuronal health.
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製品の性状

製品の状態

Liquid

補足情報

ab174417 was purified using conventional chromatography.

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一般的な情報

機能

Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins. Probably necessary for normal neurologic development.

配列の類似性

Belongs to the small Tim family.

細胞内局在性

Mitochondrion inner membrane

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製品プロトコール

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ターゲットの情報

Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins. Probably necessary for normal neurologic development.
See full target information TIMM8A
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