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

This product is a rabbit antibody that recognizes SDHAF1. The antibody CBXS-1354 can be used for immunoassay techniques such as: WB, IF.
See all SDHAF1 antibodies

Summary

Host Animal
Rabbit
Specificity
Mouse, Rat, Human
Clone
CBXS-1354
Antibody Isotype
IgG
Application
WB, IF

Basic Information

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.

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
Human644096
Mouse68332
Rat499125
UniProt ID
HumanA6NFY7
MouseQ3U276
RatB0K036
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.
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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.).

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