Dilution Information
Mouse Fumarate Hydratase/Fumarase Monoclonal Antibody
Fumarate Hydratase/Fumarase (BSB-151)
Reactivity
Human, Mouse, Rat
Synonyms
Fumarate Hydratase/Fumarase; Monoclonal Antibody; Fumarate Hydratase/Fumarase (BSB-151); fumarate hydratase; fumurase; anti-fumurate hydratase; anti-fumurase; fh; anti-fh; fumurate; fumarate; fumerate; FH; Fh1; FMRD; HLRCC; LRCC; MCL; MCUL1; Fumarate hydratase mitochondrial; anti-Fumarate Hydratase/Fumarase antibody
Host
Mouse
Reactivity
Human, Mouse, Rat
Clonality
Monoclonal
Isotype
IgG1
Clone Number
BSB-151
Form/Format
Paraffin, Frozen
Anti-fumarate hydratase is a mouse monoclonal antibody derived from cell culture supernatant that is concentrated, dialyzed, filter sterilized and diluted in buffer pH7.5, containing BSA and sodium azide as a preservative.
Anti-fumarate hydratase is a mouse monoclonal antibody derived from cell culture supernatant that is concentrated, dialyzed, filter sterilized and diluted in buffer pH7.5, containing BSA and sodium azide as a preservative.
Immunogen
Synthetic peptide corresponding to the C-terminus of the human fumarate hydratase protein.
Control
Placenta, Breast, Fallopian Tube, Colon, Kidney, Testis, Colon Adenocarcinoma, HER2 Negative Breast Cancer
Localization
Cytoplasmic
Preparation and Storage
Store at 2-8 degree C (Control Slides: Store at 20-25 degree C)
Dilution Information
Dilution Information
Immunohistochemistry (IHC)
Immunohistochemistry (IHC)
Related Product Information for anti-Fumarate Hydratase/Fumarase antibody
Fumarate hydratase (FH), or fumarase, is encoded by the FH gene, which is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, where it catalyzes the formation of L-malate from fumarate. Mutations in the FH gene can cause FH deficiency and lead to progressive encephalopathy. It was discovered that succinate dehydrogenase and FH are tumour suppressors and they are associated with metabolic dysfunction and tumorigenesis, providing biochemical evidence to explain enhanced glycolysis in tumours.
Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC) is an autosomal dominant heritable syndrome with predisposition to development of Renal Cell Carcinoma and Smooth Muscle Tumors of the skin and uterus. Cells of individuals with HLRCC had lower fumarate hydratase antibody activity than cells from normal controls, making fumarate hydratase antibody activity testing a useful method for identification and screening. Loss-of-function mutations of FH predisposes individuals to the autosomal dominant syndrome of Multiple Cutaneous and Uterine Leiomyomatosis (MCUL). Biallelic inactivation/mutations of FH are seen in 85% of Hereditary Leiomyomatosis and Renal Cell Carcinoma cases, 100% of Renal Cell Carcinoma with germline FH mutations, 19% of Papillary Renal Cell Carcinoma (Type II) have FH deficiency and 90% of FH deficiency RCC have FH mutations, 1% of unselected Leiomyomas, 2.6% of Leiomyomas in patients < 40 years old and 37- 52% of Leiomyoma with bizarre nuclei.
Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC) is an autosomal dominant heritable syndrome with predisposition to development of Renal Cell Carcinoma and Smooth Muscle Tumors of the skin and uterus. Cells of individuals with HLRCC had lower fumarate hydratase antibody activity than cells from normal controls, making fumarate hydratase antibody activity testing a useful method for identification and screening. Loss-of-function mutations of FH predisposes individuals to the autosomal dominant syndrome of Multiple Cutaneous and Uterine Leiomyomatosis (MCUL). Biallelic inactivation/mutations of FH are seen in 85% of Hereditary Leiomyomatosis and Renal Cell Carcinoma cases, 100% of Renal Cell Carcinoma with germline FH mutations, 19% of Papillary Renal Cell Carcinoma (Type II) have FH deficiency and 90% of FH deficiency RCC have FH mutations, 1% of unselected Leiomyomas, 2.6% of Leiomyomas in patients < 40 years old and 37- 52% of Leiomyoma with bizarre nuclei.
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Product Notes
The Fumarate Hydratase/Fumarase (Catalog #AAA370492) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Fumarate Hydratase/Fumarase (BSB-151) reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. It is sometimes possible for the material contained within the vial of "Fumarate Hydratase/Fumarase, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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