Fumarase (FH) Active Protein

Name: FH Active Protein; Fumarase; Research Grade; MyBioSource
Brand: MyBioSource
SKU: MBS203208
Price: 210 USD
Availability: InStock
Rating: 5 (10 reviews)

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS203208

Fumarase (Fumarate hytdratase) is an enzyme that catalyzes the reversible hydration/dehydration of fumarate to S-malate and is involved in the tricarboxylic acid (TCA), or Krebs cycle. This enzyme exists in both a cytosolic form and an N-terminal extended… More details

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Product Documentation

Sample Manual Insert View PDF

Testing Data View PDF

Certificate of Analysis (COA) View PDF

QC Report View PDF

Material Safety Data Sheet View PDF

Technical Datasheet View PDF

Protocol View PDF

Product Overview

Online Datasheet in PDF

Product Name

Fumarase (FH), Active Protein

Full Product Name

Fumarase, 44-510aa, Human, E Coli (Bioactivity Validated)

Product Gene Name

FH active protein

Product Synonym Names

Fumarate hydratase; FH; HLRCC; LRCC; MCL; MCuL1; Fumarate hydratase Fumarase; Fumarate hydratase mitochondrial; MCuL 1; Multiple hereditary cutaneous leiomyomata.

Sequence

MASQNSFRIE YDTFGELKVP NDKYYGAQTV RSTMNFKIGG VTERMPTPVI KAFGILKRAA AEVNQDYGLD PKIANAIMKA ADEVAEGKLN DHFPLVVWQT GSGTQTNMNV NEVISNRAIE MLGGELGSKI PVHPNDHVNK SQSSNDTFPT AMHIAAAIEV HEVLLPGLQK LHDALDAKSK EFAQIIKIGR THTQDAVPLT LGQEFSGYVQ QVKYAMTRIK AAMPRIYELA AGGTAVGTGL NTRIGFAEKV AAKVAALTGL PFVTAPNKFE ALAAHDALVE LSGAMNTTAC SLMKIANDIR FLGSGPRSGL GELILPENEP GSSIMPGKVN PTQCEAMTMV AAQVMGNHVA VTVGGSNGHF ELNVFKPMMI KNVLHSARLL GDASVSFTEN CVVGIQANTE RINKLMNESL MLVTALNPHI GYDKAAKIAK TAHKNGSTLK ETAIELGYLT AEQFDEWVKP KDMLGPK

Host

E Coli

Form/Format

Liquid. In 20 mM Tris-HCl Buffer (pH 8.0)

Purity/Purification

> 95% by SDS-PAGE

Concentration

1 mg/ml (determined by Bradford assay) (lot specific)

Preparation and Storage

Can be stored at 4 degree C short term (1-2 weeks).
For long term storage, aliquot and store at -20 degree C or -70 degree C.
Avoid repeated freezing and thawing cycles.

Molecular Weight

50.2kDa (467aa), confirmed by MALDI-TOF.

OMIM

U59309 mRNA

3D Structure

ModBase 3D Structure for P07954

Active Protein Note

Protein activity can be an ambiguous term. Please refer to the data sheet or inquire with Technical Support to determine the definition of activity for this product.

Antigen Species

Human

Endotoxin Level

< 1.0 EU per 1 microgram of protein (determined by LAL method)

Biological Activity

Specific activity is > 25 unit/mg, and is defined as the amount of enzyme that cleaves 1umole of L-Malate to Fumarate per minute at pH 7.5 at 37C.

Assay

1. Prepare a 180ul reaction mix into a suitable container: The concentrations are 90mM Potassium phoaphate, 90 mM L-Malic acid.
2. Equilibrate to 37C and monitor at A260nm until the value is constant using a spectrophotometer.
3. Add 20ul of recombinant Fumarase protein 50ug/ml in assay buffer.
4. Mix by inversion and record the increase at A260nm for 5minutes.
- L-Malate (Sigma-Aldrich. Cat. No, M-1000)
- 96 well UV plate (Costar, Cat. No, 3635)
- Plate reader (PerkinElmer, VICTORX3)

Product Categories/Family for FH active protein

Cell Cycle

Applications Tested/Suitable for FH active protein

SDS-PAGE

Product References and Citations for FH active protein

Lehtonen R., et al. (2003) J. Med. Genet. 40 (3): e19.; Toro JR., et al. (2003) Am. J. Hum. Genet. 73 (1): 95-106.;

Other Notes

Small volumes of FH active protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Searchable Terms for FH purchase

MBS203208 is an active protein targeting FH. Also known as Fumarase, 44-510aa, Human, E Coli (Bioactivity Validated). Host: E Coli.

Additional Details

NCBI/Uniprot data below describe general gene information for FH. It may not necessarily be applicable to this product.

NCBI GI #

19743875

NCBI GeneID

2271

NCBI Accession #

NP_000134

UniProt Primary Accession #

P07954

UniProt Secondary Accession #

B1ANK7

UniProt Related Accession #

P07954

GenBank Nucleotide #

NM_000143.3

NCBI Official Full Name

fumarate hydratase, mitochondrial

NCBI Official Synonym Full Names

fumarate hydratase

NCBI Official Symbol

NCBI Official Synonym Symbols

MCL; FMRD; LRCC; HLRCC; MCUL1

UniProt Protein Name

Fumarate hydratase, mitochondrial

Protein Family

Fumarate hydratase

UniProt Gene Name

UniProt Synonym Gene Names

Fumarase

NCBI Protein Information

fumarate hydratase, mitochondrial

NCBI Summary for FH

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]

UniProt Comments for FH

Also acts as a tumor suppressor.MiscellaneousThere are 2 substrate-binding sites: the catalytic A site, and the non-catalytic B site that may play a role in the transfer of substrate or product between the active site and the solvent. Alternatively, the B site may bind allosteric effectors ().

Research Articles on FH

1. A novel mutation in FH gene (c.1349_1352delATGA) in hereditary leiomyomatosis and renal cell cancer syndrome

Precautions

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

Disclaimer

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Products associated with FH active protein

Reference Product	PubMed Publications
SDHB active protein	>35 publications with FH and SDHB
SDHC active protein	>18 publications with FH and SDHC
SDHA active protein	>16 publications with FH and SDHA
MDH2 active protein	>8 publications with FH and MDH2
MDH1 active protein	>2 publications with FH and MDH1
ME1 active protein	>1 publications with FH and ME1

Pathways associated with FH active protein

Products by Pathway	Pathway Diagram
Amino Acid Metabolism Pathway antibodies	Amino Acid Metabolism Pathway Diagram
Carbon Metabolism Pathway antibodies	Carbon Metabolism Pathway Diagram
Carbon Metabolism Pathway antibodies	Carbon Metabolism Pathway Diagram
Citrate Cycle (TCA Cycle) Pathway antibodies	Citrate Cycle (TCA Cycle) Pathway Diagram
Citrate Cycle (TCA Cycle) Pathway antibodies	Citrate Cycle (TCA Cycle) Pathway Diagram
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway antibodies	Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway Diagram
Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway antibodies	Citrate Cycle (TCA Cycle, Krebs Cycle) Pathway Diagram
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway antibodies	Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway Diagram
Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway antibodies	Citrate Cycle, Second Carbon Oxidation, 2-oxoglutarate => Oxaloacetate Pathway Diagram
Citric Acid Cycle (TCA Cycle) Pathway antibodies	Citric Acid Cycle (TCA Cycle) Pathway Diagram

Diseases associated with FH active protein

Disease Name	Pubmed Publications
Dyslipidemias Antibodies	>2168 publications with FH and Dyslipidemias
Heart Diseases Antibodies	>664 publications with FH and Heart Diseases
Nervous System Diseases Antibodies	>439 publications with FH and Nervous System Diseases
Kidney Diseases Antibodies	>358 publications with FH and Kidney Diseases
Hypertension Antibodies	>341 publications with FH and Hypertension
Brain Diseases Antibodies	>288 publications with FH and Brain Diseases
Inflammation Antibodies	>201 publications with FH and Inflammation
Adenocarcinoma Antibodies	>138 publications with FH and Adenocarcinoma
Pain Antibodies	>129 publications with FH and Pain
Lung Diseases Antibodies	>117 publications with FH and Lung Diseases

Organs/Tissues associated with FH active protein

Organ/Tissue Name	Pubmed Publications
Blood Antibodies	>2695 publications with FH and Blood
Heart Antibodies	>1052 publications with FH and Heart
Vascular Antibodies	>622 publications with FH and Vascular
Liver Antibodies	>425 publications with FH and Liver
Bone Antibodies	>401 publications with FH and Bone
Brain Antibodies	>375 publications with FH and Brain
Kidney Antibodies	>368 publications with FH and Kidney
Skin Antibodies	>269 publications with FH and Skin
Muscle Antibodies	>226 publications with FH and Muscle
Lung Antibodies	>183 publications with FH and Lung