Fumarate Hydratase (FH) (AA 33-511) antibody

Details for Product No. ABIN393935
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Antigen
Synonyms FH, DDBDRAFT_0205237, DDBDRAFT_0231397, DDBDRAFT_0231400, DDB_0205237, DDB_0231397, DDB_0231400, HLRCC, LRCC, MCL, MCUL1, Fh1, im:7152785, ns:zf-e152, zf-e152, zgc:66253, zgc:77498, Fh, Fh-1
Epitope
AA 33-511
(12), (7), (6), (6), (5), (2), (2), (1), (1), (1), (1), (1)
Reactivity
Human
(50), (24), (19), (19), (16), (12), (1)
Host
Mouse
(39), (17), (7)
Clonality (Clone)
Monoclonal ()
Conjugate
Un-conjugated
(5), (2), (2), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Western Blotting (WB)
(51), (34), (21), (14), (10), (7), (6), (5), (3), (3), (3), (3), (2)
Pubmed 5 references available
Quantity 0.1 mg
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Catalog No. ABIN393935
450.00 $
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Immunogen FH (AAH03108, 33 a.a. ~ 511 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 K
Clone 3E7
Isotype IgG2b kappa
Specificity FH (AAH03108, 33 a.a. ~ 511 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Purification This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Alternative Name FH
Background Synonyms: Serine protease inhibitor J6, 47 kDa heat shock protein, Collagen-binding protein, Colligin, Serpin H1,Hsp47, Cbp1, Serpinh1
Molecular Weight 54637 DA
Gene ID 12406
Research Area Translation Factors, Signaling, Cell Structure
Application Notes Western blot = 1:500-1000
Comment

Background: 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.

Restrictions For Research Use only
Concentration 0.5mg/ml
Buffer PBS with 0.09% (w/v) sodium azide.
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage 4 °C/-20 °C
Storage Comment Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles
Expiry Date 6 months
Supplier Images
anti-Fumarate Hydratase (FH) (AA 33-511) antibody FH monoclonal antibody (M09), clone 3E7 Western Blot analysis of FH expression in HeLa on formalin-fixed paraffin-embedded human seminoma (antibody concentration 3 µg/mL).
anti-Fumarate Hydratase (FH) (AA 33-511) antibody (2) anti-Fumarate Hydratase (FH) (AA 33-511) antibody (Image 2)
Background publications Tian, Liu, Usa et al.: "Novel role of fumarate metabolism in dahl-salt sensitive hypertension." in: Hypertension, Vol. 54, Issue 2, pp. 255-60, 2009 (PubMed).

McCauley, Zuvich, Bradford et al.: "Follow-up examination of linkage and association to chromosome 1q43 in multiple sclerosis." in: Genes and immunity, Vol. 10, Issue 7, pp. 624-30, 2009 (PubMed).

Yang, Valera, Padilla-Nash et al.: "UOK 262 cell line, fumarate hydratase deficient (FH-/FH-) hereditary leiomyomatosis renal cell carcinoma: in vitro and in vivo model of an aberrant energy metabolic pathway in human cancer." in: Cancer genetics and cytogenetics, Vol. 196, Issue 1, pp. 45-55, 2009 (PubMed).

Yogev, Yogev, Singer et al.: "Fumarase: a mitochondrial metabolic enzyme and a cytosolic/nuclear component of the DNA damage response." in: PLoS biology, Vol. 8, Issue 3, pp. e1000328, 2010 (PubMed).

Shimada, Miyagawa, Kawashima et al.: "An approach based on a genome-wide association study reveals candidate loci for narcolepsy." in: Human genetics, Vol. 128, Issue 4, pp. 433-41, 2010 (PubMed).

Validation Images
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