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AB174417

Recombinant Human TIMM8A/DDP protein

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Recombinant Human TIMM8A/DDP protein 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.

View Alternative Names

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 (AB174417)
  • SDS-PAGE

Supplier Data

SDS-PAGE - Recombinant Human TIMM8A/DDP protein (AB174417)

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

Key facts

Purity

>90% SDS-PAGE

Expression system

Escherichia coli

Tags

His tag N-Terminus

Applications

SDS-PAGE, Mass Spec

applications

Biologically active

No

Accession

O60220

Animal free

No

Carrier free

No

Species

Human

Storage buffer

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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Product details

This product was previously labelled as TIMM8A
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Sequence info

[{"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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Properties and storage information

Shipped at conditions
Blue Ice
Appropriate short-term storage duration
1-2 weeks
Appropriate short-term storage conditions
+4°C
Appropriate long-term storage conditions
-20°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.

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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Specifications

Form

Liquid

Additional notes

ab174417 was purified using conventional chromatography.

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General info

Function

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.

Sequence similarities

Belongs to the small Tim family.

Subcellular localisation

Mitochondrion inner membrane

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Product protocols

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Target data

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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