Product info:

The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.

Alternative names:

FH; FMRD; HLRCC; LRCC; MCL; MCUL1

Species reactivity:

Human, Mouse, Rat

Host:

Rabbit

Cellular localisation:

Cytoplasm, Mitochondrion

Isotype:

IgG

Immunogen:

Recombinant fusion protein containing a sequence corresponding to amino acids 1-280 of human FH (NP_000134.2).

Positive control:

U-87MG, LO2, 22Rv1, NCI-H460, HL-60, NIH/3T3, Mouse heart, Mouse kidney, Rat liver

AA Sequence:

MYRALRLLARSRPLVRAPAAALASAPGLGGAAVPSFWPPNAARMASQNSFRIEYDTFGELKVPNDKYYGAQTVRSTMNFKIGGVTERMPTPVIKAFGILKRAAAEVNQDYGLDPKIANAIMKAADEVAEGKLNDHFPLVVWQTGSGTQTNMNVNEVISNRAIEMLGGELGSKIPVHPNDHVNKSQSSNDTFPTAMHIAAAIEVHEVLLPGLQKLHDALDAKSKEFAQIIKIGRTHTQDAVPLTLGQEFSGYVQQVKYAMTRIKAAMPRIYELAAGGTAVG

Purification:

Affinity purification

Molecular weight:

Calculated MW: 50kDa/54kDa Observed MW: 50kDa

Form:

Liquid

Applications:

Request info at info@sobekbio.com

Dilution:

WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:100

Storage buffer:

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Storage:

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

More info:

Email: info@sobekbio.com

Orders:

Email: orders@sobekbio.com