Technical Data

S0553-27
Clone Type
Polyclonal
Host
Goat
Source
Human
Isotype
IgG
Grade
Affinity Purified
Applications
E WB
Crossreactivity
Hu
Accession #
NP_001036096.1
Shipping Temp
Blue Ice
Storage Temp
-20°C
Goat Anti-Succinate Dehydrogenase Complex Assembly Factor 1, SDHAF1
SDHAF1, succinate dehydrogenase complex assembly factor 1

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).

Applications
Suitable for use in ELISA and Western Blot. Other applications have not been tested.
Recommended Dilutions
Peptide ELISA Titer: 1:16,000 Western Blot: 0.3-1ug/ml detects ~19kD band observed in Human Brain (Cerebellum) lysates (calculated MW of 12.8kD according to NP_001036096.1). Optimal dilutions to be determined by the researcher.
Storage and Stability
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Immunogen
Synthetic peptide corresponding to C-HDSTGAPETRPDGR, from the C-Terminus of human SDHAF1 (NP_001036096.1).
Form
Supplied as a liquid in Tris saline, pH 7.2, 0.5% BSA, 0.02% sodium azide.
Purity
Purified by peptide affinity chromatography.
Specificity
Recognizes human SDHAF1. Species sequence homology: mouse and canine.

Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.

References
1. Ghezzi D, Goffrini P, Uziel G, Horvath R, Klopstock T, Lochmüller H, D\1Adamo P, Gasparini P, Strom TM, Prokisch H, Invernizzi F, Ferrero I, Zeviani M. SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy. Nat Genet. 2009 May 24.
USBio References
No references available
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