SDHAF1 Antibody, FITC conjugated

Datasheet

Code	CSB-PA406800LC01HU
Size	US$166
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Product Details
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) SDHAF1 Polyclonal antibody

Uniprot No.

A6NFY7

Target Names

SDHAF1

Alternative Names

LYR motif containing 8 antibody; LYR motif-containing protein 8 antibody; LYRM8 antibody; mitochondrial antibody; SDH assembly factor 1 antibody; SDHAF1 antibody; SDHF1_HUMAN antibody; Succinate dehydrogenase assembly factor 1 antibody; Succinate dehydrogenase assembly factor 1 mitochondrial antibody; Succinate dehydrogenase complex assembly factor 1 antibody

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human Succinate dehydrogenase assembly factor 1, mitochondrial protein (78-115AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

FITC

Clonality

Polyclonal

Isotype

IgG

Purification Method

>95%, Protein G purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4

Form

Liquid

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function

Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF3. Contributes to iron-sulfur cluster incorporation into SDHB by binding to SDHB and recruiting the iron-sulfur transfer complex formed by HSC20, HSPA9 and ISCU through direct binding to HSC20.

Gene References into Functions

that SDHAF1 contributes to iron-sulfur (Fe-S) cluster incorporation into the Fe-S subunit of CII, SDHB. PMID: 26749241
Studies indicate that an array of tumor syndromes caused by complex II-associated mutations in genes SDHA, SDHB, SDHC, SDHD, SDHAF1 and SDHAF2 have been identified over a decade. PMID: 23174333
Leukoencephalopathy with accumulated succinate is a key symptom of defective complex II assembly due to SDHAF1 mutations PMID: 22995659

Involvement in disease

Mitochondrial complex II deficiency (MT-C2D)

Subcellular Location

Mitochondrion matrix.

Protein Families

Complex I LYR family, SDHAF1 subfamily

Tissue Specificity

Ubiquitously expressed.

Database Links

HGNC: 33867

OMIM: 252011

KEGG: hsa:644096

STRING: 9606.ENSP00000368165

UniGene: Hs.356460

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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)

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Address

7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA

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