Product: FH Antibody
Catalog: DF7460
Source: Rabbit
Application: WB, IHC
Reactivity: Human, Mouse, Rat
Prediction: Pig, Zebrafish, Bovine, Horse, Rabbit, Dog
Mol.Wt.: 54kDa; 55kD(Calculated).
Uniprot: P07954
RRID: AB_2839397

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Product Info

Source:
Rabbit
Application:
WB 1:500-1:2000, IHC 1:50-1:200
*The optimal dilutions should be determined by the end user.
*Tips:

WB: For western blot detection of denatured protein samples. IHC: For immunohistochemical detection of paraffin sections (IHC-p) or frozen sections (IHC-f) of tissue samples. IF/ICC: For immunofluorescence detection of cell samples. ELISA(peptide): For ELISA detection of antigenic peptide.

Reactivity:
Human,Mouse,Rat
Prediction:
Pig(100%), Zebrafish(100%), Bovine(83%), Horse(100%), Rabbit(100%), Dog(100%)
Clonality:
Polyclonal
Specificity:
FH Antibody detects endogenous levels of total FH.
RRID:
AB_2839397
Cite Format: Affinity Biosciences Cat# DF7460, RRID:AB_2839397.
Conjugate:
Unconjugated.
Purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin (Thermo Fisher Scientific).
Storage:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. Store at -20 °C. Stable for 12 months from date of receipt.
Alias:

Fold/Unfold

FH; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; Fumarate hydratase, mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; MS709; Multiple hereditary cutaneous leiomyomata;

Immunogens

Immunogen:
Uniprot:
Gene(ID):
Expression:
P07954 FUMH_HUMAN:

Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology.

Description:
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.
Sequence:
MYRALRLLARSRPLVRAPAAALASAPGLGGAAVPSFWPPNAARMASQNSFRIEYDTFGELKVPNDKYYGAQTVRSTMNFKIGGVTERMPTPVIKAFGILKRAAAEVNQDYGLDPKIANAIMKAADEVAEGKLNDHFPLVVWQTGSGTQTNMNVNEVISNRAIEMLGGELGSKIPVHPNDHVNKSQSSNDTFPTAMHIAAAIEVHEVLLPGLQKLHDALDAKSKEFAQIIKIGRTHTQDAVPLTLGQEFSGYVQQVKYAMTRIKAAMPRIYELAAGGTAVGTGLNTRIGFAEKVAAKVAALTGLPFVTAPNKFEALAAHDALVELSGAMNTTACSLMKIANDIRFLGSGPRSGLGELILPENEPGSSIMPGKVNPTQCEAMTMVAAQVMGNHVAVTVGGSNGHFELNVFKPMMIKNVLHSARLLGDASVSFTENCVVGIQANTERINKLMNESLMLVTALNPHIGYDKAAKIAKTAHKNGSTLKETAIELGYLTAEQFDEWVKPKDMLGPK

Predictions

Predictions:

Score>80(red) has high confidence and is suggested to be used for WB detection. *The prediction model is mainly based on the alignment of immunogen sequences, the results are for reference only, not as the basis of quality assurance.

Species
Results
Score
Pig
100
Horse
100
Dog
100
Zebrafish
100
Rabbit
100
Bovine
83
Sheep
0
Xenopus
0
Chicken
0
Model Confidence:
High(score>80) Medium(80>score>50) Low(score<50) No confidence

PTMs - P07954 As Substrate

Site PTM Type Enzyme
S46 Phosphorylation
Y54 Phosphorylation
K61 Acetylation
K61 Ubiquitination
K66 Acetylation
K66 Ubiquitination
Y67 Phosphorylation
Y68 Phosphorylation
S75 Phosphorylation
K80 Acetylation
T90 Phosphorylation
K94 Acetylation
K94 Ubiquitination
K100 Methylation
K100 Ubiquitination
Y110 Phosphorylation
K115 Acetylation
K115 Ubiquitination
K122 Acetylation
S171 Phosphorylation
K172 Acetylation
K172 Ubiquitination
K221 Ubiquitination
K223 Ubiquitination
K230 Ubiquitination
T236 Phosphorylation P78527 (PRKDC)
Y251 Phosphorylation
K256 Acetylation
K292 Acetylation
K292 Ubiquitination
T301 Phosphorylation
R343 Methylation
S351 Phosphorylation
S366 Phosphorylation
Y465 Phosphorylation
K467 Acetylation
K470 Acetylation
K473 Acetylation
K477 Acetylation
Y491 Phosphorylation
T493 Phosphorylation
K502 Ubiquitination

Research Backgrounds

Function:

Catalyzes the reversible stereospecific interconversion of fumarate to L-malate. Experiments in other species have demonstrated that specific isoforms of this protein act in defined pathways and favor one direction over the other (Probable).

Catalyzes the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH.

Catalyzes the dehydration of L-malate to fumarate (By similarity). Fumarate metabolism in the cytosol plays a role during urea cycle and arginine metabolism; fumarate being a by-product of the urea cycle and amino-acid catabolism (By similarity). Also plays a role in DNA repair by promoting non-homologous end-joining (NHEJ). In response to DNA damage and phosphorylation by PRKDC, translocates to the nucleus and accumulates at DNA double-strand breaks (DSBs): acts by catalyzing formation of fumarate, an inhibitor of KDM2B histone demethylase activity, resulting in enhanced dimethylation of histone H3 'Lys-36' (H3K36me2).

PTMs:

Phosphorylation at Thr-236 by PRKDC in response to DNA damage promotes translocation to the nucleus and recruitment to DNA double-strand breaks (DSBs).

Subcellular Location:

Mitochondrion.

Cytoplasm>Cytosol. Nucleus. Chromosome.
Note: Translocates to the nucleus in response to DNA damage: localizes to DNA double-strand breaks (DSBs) following phosphorylation by PRKDC.

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionSubcellular location
Tissue Specificity:

Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology.

Subunit Structure:

Homotetramer. Interacts with H2AZ1.

Family&Domains:

Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.

Research Fields

· Human Diseases > Cancers: Overview > Pathways in cancer.   (View pathway)

· Human Diseases > Cancers: Specific types > Renal cell carcinoma.   (View pathway)

· Metabolism > Carbohydrate metabolism > Citrate cycle (TCA cycle).

· Metabolism > Carbohydrate metabolism > Pyruvate metabolism.

· Metabolism > Global and overview maps > Metabolic pathways.

· Metabolism > Global and overview maps > Carbon metabolism.

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