Rabbit Anti-SDHAF1 Recombinant Antibody (CBXS-1354) (CBMAB-S4212-CQ)

Name: Rabbit Anti-SDHAF1 Recombinant Antibody (CBXS-1354)
SKU: CBMAB-S4212-CQ
Rating: 5 (1 reviews)

See all SDHAF1 antibodies

Compare Online Inquiry

Datasheet

SDS

Request for COA

Datasheet Target Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Rabbit

Clone

CBXS-1354

Application

WB, IF

Specificity

Mouse, Rat, Human

Antibody Isotype

IgG

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Concentration

1.262 mg/mL

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

Succinate Dehydrogenase Complex Assembly Factor 1

Introduction

The succinate dehydrogenase (SDH) complex (or complex II) of the mitochondrial respiratory chain is composed of 4 individual subunits. The protein encoded by this gene resides in the mitochondria, and is essential for SDH assembly, but does not physically associate with the complex in vivo. Mutations in this gene are associated with SDH-defective infantile leukoencephalopathy (mitochondrial complex II deficiency).

Entrez Gene ID

Human	644096
Mouse	68332
Rat	499125

UniProt ID

Human	A6NFY7
Mouse	Q3U276
Rat	B0K036

Alternative Names

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;

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 (PubMed:24954417, PubMed:19465911).
Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants (PubMed:24954417).
May act together with SDHAF3 (PubMed:24954417).
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 (PubMed:26749241).

Biological Process

Mitochondrial respiratory chain complex II assembly

Cellular Location

Mitochondrion matrix

Involvement in disease

Mitochondrial complex II deficiency, nuclear type 2 (MC2DN2):
A form of mitochondrial complex II deficiency, a disorder with heterogeneous clinical manifestations. Some patients have multisystem involvement of the brain, heart, muscle, liver, and kidneys resulting in death in infancy, whereas others have only isolated cardiac or muscle involvement with onset in adulthood and normal cognition. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome. MC2DN2 inheritance is autosomal recessive.

Ask a question We look forward to hearing from you.

0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Have you used Rabbit Anti-SDHAF1 Recombinant Antibody (CBXS-1354)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon

$30 eGift Card
Submit a review

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Request bulk or custom quote Second antibody and isotype control

Contact us

Tel: (USA)
(UK)
Fax:

Global Locations

Email:

Online Inquiry