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Products:Signal Transduction >> Metabolism >> Energy Metabolism
Overview
Product name
Anti-FH antibody
See all FH products (7) ...
Description
Goat polyclonal to FH
Tested applications
WB, ELISAmore details
Cross reactivity
Reacts with
Human
Predicted to work with
Mouse, Rat, Horse, Cow, Pig, Fruit fly (Drosophila melanogaster), Zebrafish, a wide range of other species, Cynomolgus Monkey, Chlamydia trachomatis, Orangutan
Immunogen
Synthetic peptide: C-HPNDHVNKSQSSND, corresponding to internal sequence amino acids 176-189 of Human FH
C-HPNDHVNK SQSSND
Positive control
Human Kidney, Liver and Lung lysates
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage buffer
Preservative: 0.02% Sodium Azide
Constituents: 0.5% BSA, Tris buffered saline, pH 7.3
Concentration
Concentration information loading...
Purity
Immunogen affinity purified
Purification notes
Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Clonality
Polyclonal
Isotype
IgG
Research areas
Cancer >> Cancer Metabolism >> Metabolic signaling pathway >> Integration of energy metabolism
Cancer >> Oncoproteins/suppressors >> Tumor suppressors >> Other
Signal Transduction >> Metabolism >> Energy Metabolism
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Western blot - FH antibody (ab62825)
(enlarge)
Applications
Show applications key
Our Abpromise guarantee covers the use of ab62825 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
WB: Use a concentration of 0.01 - 0.03 µg/ml.Detects a band of approximately 48 kDa (predicted molecular weight: 55 kDa).
ELISA
ELISA: 1/64000ELISA: 1/64000
Target
Function
Also acts as a tumor suppressor.
Pathway
Carbohydrate metabolism; tricarboxylic acid cycle; (S)-malate from fumarate: step 1/1.
Involvement in disease
Defects in FH are the cause of fumarase deficiency (FHD) [MIM:606812]; also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.
Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids).
Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].
Sequence similarities
Belongs to the class-II fumarase/aspartase family. Fumarase subfamily.
Cellular localization
Cytoplasm and Mitochondrion.
Target information above from: UniProt accessionP07954
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- FH antibody
- Fumarase antibody
- Fumarate hydratase mitochondrial antibody
see all
Database links
- Entrez Gene: 520260 Cow
- Entrez Gene: 2271 Human
- Entrez Gene: 14194 Mouse
- Entrez Gene: 24368 Rat
- Entrez Gene: 393938 Zebrafish
- Omim: 136850 Human
- SwissProt: P07954 Human
- SwissProt: P97807 Mouse
see all
Anti-FH antibody images:
Western blot - FH antibody (ab62825)
Anti-FH antibody (ab62825) at 0.01 µg/ml + Human Kidney lysate in RIPA buffer at 35 µg/ml
Predicted band size : 55 kDa
Observed band size : 48 kDa (why is the actual band size different from the predicted?)
Detected by chemiluminescence
References for Anti-FH antibody (ab62825)
This product has been referenced in:
- Kienhöfer Jet al. Association of mitochondrial antioxidant enzymes with mitochondrial DNA as integral nucleoid constituents. FASEB J 23:2034-44 (2009). WB; Human.Read more (PubMed: 19228881) »
See 1 publication for this product
Publishing research using ab62825? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"