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AB174417

重组人 TIMM8A/DDP 蛋白 (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.

查看别名

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

1 Images
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)

关键信息

纯度

>90% SDS-PAGE

表达系统

Escherichia coli

标签

His tag N-Terminus

应用

SDS-PAGE, Mass Spec

applications

生物活性

No

访问

O60220

不含动物源

No

不含载体蛋白

No

种属

Human

存储溶液

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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反应性数据

{ "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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产品详情

This product was previously labelled as TIMM8A
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序列信息

[{"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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性能和储存信息

运输条件
Blue Ice
推荐的短期储存时间
1-2 weeks
推荐的短期储存条件
+4°C
推荐的长期储存条件
-20°C
分装信息
Upon delivery aliquot
储存信息
Avoid freeze / thaw cycle
False
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补充信息

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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特殊说明

形式

Liquid

附加说明

ab174417 was purified using conventional chromatography.

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常规信息

功能

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.

序列相似性

Belongs to the small Tim family.

亚细胞定位

Mitochondrion inner membrane

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产品实验方案

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靶点信息

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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