SDHAF

SDHAF

SDHAF

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  • View Data Sheet

    Description:

    Succinate Dehydrogenase Complex Assembly Factor 2 Human Recombinant

    Succinate dehydrogenase assembly factor 2 mitochondrial, SDH assembly factor 2, Succinate dehydrogenase subunit 5, mitochondrial, hSDH5, SDHAF2, C11orf79, PGL2, SDH5.

    Product # :

    PRO-593

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    • description
    • source
    • formulation
    • purity
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    Description

    SDHAF2 Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 160 amino acids (30-166 a.a) and having a molecular mass of 19kDa.SDHAF2 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

    Source

    Escherichia Coli.

    Formulation

    SDHAF2 protein solution (0.5mg/ml) containing 20mM Tris-HCl buffer (pH 8.0), 0.2M NaCl, 30% glycerol and 1mM DTT.

    Purity

    Greater than 90.0% as determined by SDS-PAGE.

    More Info

    • Introduction

      Succinate Dehydrogenase Complex Assembly Factor 2 (SDHAF2) is a mitochondrial protein required for the flavination of a succinate dehydrogenase complex subunit essential for activity of the complex. DHAF2 is necessary for insertion of FAD cofactor into SDHA, the catalytic subunit of succinate dehydrogenase (SDH). SDH is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). SDHAF2 gene defects are the cause of hereditary paragangliomas type 2 (PGL2), otherwise known as familial non-chromaffin paragangliomas type 2.

    • Synonyms

      Succinate dehydrogenase assembly factor 2 mitochondrial, SDH assembly factor 2, Succinate dehydrogenase subunit 5, mitochondrial, hSDH5, SDHAF2, C11orf79, PGL2, SDH5.

    • Physical Appearance

      Sterile Filtered colorless solution.

    • Stability

      Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.

    • Amino Acid Sequence

      MGSSHHHHHH SSGLVPRGSH MGSSFRRFYR GDSPTDSQKD MIEIPLPPWQ ERTDESIETK RARLLYESRK RGMLENCILL SLFAKEHLQH MTEKQLNLYD RLINEPSNDW DIYYWATEAK PAPEIFENEV MALLRDFAKN KNKEQRLRAP DLEYLFEKPR.

    ProSpec's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.

    Sdhaf2 Human
  • View Data Sheet

    Description:

    Succinate Dehydrogenase Complex Assembly Factor 1 Human Recombinant

    Succinate Dehydrogenase Complex Assembly Factor 1, LYR Motif-Containing Protein 8, LYR Motif Containing 8, SDH Assembly Factor 1, LYRM8, Succinate Dehydrogenase Assembly Factor 1, Mitochondrial, Succinate dehydrogenase assembly factor 1, mitochondrial.

    Product # :

    PRO-2123

    Price :

    Quantity :

    Shipping Method :

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    Shipped with Ice Packs

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    • description
    • source
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    • More Info

    Description

    SDHAF1 Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 138 amino acids (1-115 a.a) and having a molecular mass of 15.2kDa. SDHAF1 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

    Source

    Escherichia Coli.

    Formulation

    SDHAF1 protein solution (0.25mg/ml) containing Phosphate buffered saline (pH7.4), 30% glycerol, 2mM DTT and 0.1mM PMSF.

    Purity

    Greater than 85.0% as determined by SDS-PAGE.

    More Info

    • Introduction

      Succinate dehydrogenase assembly factor 1 (SDHAF1) is a member of the complex I LYR family. SDHAF1 plays an important role in succinate dehydrogenase complex (SDH) assembly, a complex which is involved in complex II of the mitochondrial electron transport chain. SDHAF1 functions through taking part in mitochondrial biosynthesis of iron-sulfur centers for complex II.

    • Synonyms

      Succinate Dehydrogenase Complex Assembly Factor 1, LYR Motif-Containing Protein 8, LYR Motif Containing 8, SDH Assembly Factor 1, LYRM8, Succinate Dehydrogenase Assembly Factor 1, Mitochondrial, Succinate dehydrogenase assembly factor 1, mitochondrial.

    • Physical Appearance

      Sterile Filtered colorless solution.

    • Stability

      Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.

    • Amino Acid Sequence

      MGSSHHHHHH SSGLVPRGSH MGSMSRHSRL QRQVLSLYRD LLRAGRGKPG AEARVRAEFR QHAGLPRSDV LRIEYLYRRG RRQLQLLRSG HATAMGAFVR PRAPTGEPGG VGSQPDDGDS PRNPHDSTGA PETRPDGR.

    ProSpec's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.

    Sdhaf1 Human

About SDHAF / Succinate Dehydrogenase Complex Assembly Factor:

Succinate dehydrogenase complex assembly factor 1 (SDHAF1) is a chaperone protein used in the assembly of the succinate dehydrogenase (SDH) complex (complex II). It is also known as LYR motif-containing protein 8 (LYRM8).
Mutations in SDHAF1 are strongly linked with mitochondrial complex II deficiency. Although this is a disorder of the mitochondrial respiratory chain it has a wide range of psychomotor manifestations. SDHAF1 mutations are also strongly linked with SDH-defective infantile leukoencephalopathy.

SDHAF Function
The SDHAF1 protein shields SDHB from the oxidants which could otherwise damage it. This enables the assembly and stabilization of the succinate dehydrogenase (SDH) complex (complex II). SDH is necessary for both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain.
It also binds directly to the co-chaperone protein HSC20. This enables the scaffold protein ISCU to transfer the Fe-S prosthetic group to recipient apoproteins (namely SDHB). Indirectly, therefore, the SDHAF1 protein makes it possible for SDHB to acquire Fe-S clusters.

Succinate Dehydrogenase Complex Assembly Factor Structure
The SDHAF1 gene is located in position 13.12 of the q arm of chromosome 19 (19q13.12). It has 1 exon. The SDHAF1 protein is a 12.8 kDa protein. It consists of 115 amino acids.

SDHAF Interaction
SDHAF1 has a total of 27 protein-protein interactions. Of these, 15 are co-complex interactions. Genes known to interact with SDHAF1 include:
ccdc136
CIDEB
HSCB
HSPA9
ISCU
KRT27
SDHB

Succinate Dehydrogenase Complex Assembly Factor Mechanism of action
An arginine-rich region in the C terminus of SDHAF1 creates a transient bond with the aromatic peptides of SDHB. This engages specifically with an Fe-S donor complex through an LYR motif near its N-terminal domain. The scaffold is holo-ISCU, and the co-chaperone-chaperone pair is HSC20-HSPA9.