Product Name	Fumarase/FH
Chinese Name	富马酸水合酶单克隆抗体
Alias	FH; Fumarase; Fumarate hydratase; Fumarate hydratase mitochondrial; FUMH_HUMAN; HLRCC; LRCC; MCL; MCUL 1; MCUL1; Multiple hereditary cutaneous leiomyomata.
Research Area	Tumour  Cell biology  immunology  transcriptional regulatory factor  Mitochondrion
Immunogen Species	Mouse
Clonality	Monoclonal
Clone NO.	4C12
React Species	Human, Rat,  (predicted: Mouse, )
Applications	IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	51kDa
Cellular localization	cytoplasmic Mitochondrion
Form	Liquid
Concentration	1mg/ml
immunogen	Recombinant human Fumarase/FH Protein
Lsotype	IgG
Purification	affinity purified by Protein G
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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. [provided by RefSeq, Jul 2008] Function: Also acts as a tumor suppressor. Subunit: Homotetramer. Subcellular Location: Cytoplasm, Mitochondrion Tissue Specificity: Expressed in red blood cells; underexpressed in red blood cells (cytoplasm) of patients with hereditary non-spherocytic hemolytic anemia of unknown etiology. DISEASE: Fumarase deficiency (FMRD): The disease is caused by mutations affecting the gene represented in this entry. A severe autosomal recessive metabolic disorder characterized by early-onset hypotonia, profound psychomotor retardation, and brain abnormalities, such as agenesis of the corpus callosum, gyral defects, and ventriculomegaly. Many patients show neonatal distress, metabolic acidosis, and/or encephalopathy. Hereditary leiomyomatosis and renal cell cancer (HLRCC): The disease is caused by mutations affecting the gene represented in this entry. A disorder characterized by predisposition to cutaneous and uterine leiomyomas, and papillary type 2 renal cancer which occurs in about 20% of patients. Similarity: Belongs to the class-II fumarase/aspartase family. Fumarase subfamily. SWISS: P07954 Gene ID: 2271 Database links: Entrez Gene: 520260 Cow Entrez Gene: 480092 Dog Entrez Gene: 2271 Human Entrez Gene: 14194 Mouse Entrez Gene: 100627128 Pig Entrez Gene: 24368 Rat Entrez Gene: 393938 Zebrafish Omim: 136850 Human SwissProt: P07954 Human SwissProt: P97807 Mouse SwissProt: P10173 Pig SwissProt: P14408 Rat SwissProt: Q7SX99 Zebrafish Unigene: 592490 Human Unigene: 41502 Mouse Unigene: 29782 Rat Unigene: 104452 Zebrafish
Product Picture	Paraformaldehyde-fixed, paraffin embedded (human myocardium); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (human gastric carcinoma); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (mouse heart); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (rat kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (human kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (human gastric carcinoma); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (human kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (human myocardium); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Fumarase/FH) Monoclonal Antibody, Unconjugated (SLM-33167M) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Mouse)(sp-0024) instructionsand DAB staining.

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