Mouse Anti-GATM Recombinant Antibody (2H7) (CBMAB-G2330-LY)

Name: Mouse Anti-GATM Recombinant Antibody (2H7)
SKU: CBMAB-G2330-LY
Rating: 5 (1 reviews)

See all GATM antibodies

Compare Online Inquiry

Datasheet

SDS

Request for COA

Datasheet Target Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

2H7

Application

ELISA, WB

Specificity

Human

Antibody Isotype

IgM, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

Target

Full Name

Glycine Amidinotransferase

Introduction

This gene encodes a mitochondrial enzyme that belongs to the amidinotransferase family. This enzyme is involved in creatine biosynthesis, whereby it catalyzes the transfer of a guanido group from L-arginine to glycine, resulting in guanidinoacetic acid, the immediate precursor of creatine. Mutations in this gene cause arginine:glycine amidinotransferase deficiency, an inborn error of creatine synthesis characterized by cognitive disability, language impairment, and behavioral disorders. [provided by RefSeq, Jul 2008]

Entrez Gene ID

2628

UniProt ID

P50440

Alternative Names

Glycine Amidinotransferase; L-Arginine:Glycine Amidinotransferase; Transamidinase; EC 2.1.4.1; AGAT; Glycine Amidinotransferase (L-Arginine:Glycine Amidinotransferase);

Function

Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.

Biological Process

Creatine biosynthetic process Source: MGI
Creatine metabolic process Source: CAFA
Learning or memory Source: CAFA
Multicellular organism development Source: CAFA
Muscle atrophy Source: CAFA
Positive regulation of cold-induced thermogenesis Source: YuBioLab

Cellular Location

Isoform 1: Mitochondrion inner membrane. Probably attached to the outer side of the inner membrane.
Isoform 2: Cytoplasm

Involvement in disease

Cerebral creatine deficiency syndrome 3 (CCDS3):
An autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of expressive and cognitive speech, and severe depletion of creatine/phosphocreatine in the brain. Most patients develop a myopathy characterized by muscle weakness and atrophy later in life.
Fanconi renotubular syndrome 1 (FRTS1):
A form of Fanconi renotubular syndrome, a disease due to a generalized dysfunction of the proximal kidney tubule resulting in decreased solute and water reabsorption. Patients have polydipsia and polyuria with phosphaturia, glycosuria and aminoaciduria. They may develop hypophosphatemic rickets or osteomalacia, acidosis and a tendency toward dehydration. Some eventually develop renal insufficiency. FRTS1 inheritance is autosomal dominant.

Ask a question We look forward to hearing from you.

0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Have you used Mouse Anti-GATM Recombinant Antibody (2H7)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon

$30 eGift Card
Submit a review

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Related Products

Mouse Anti-GATM Recombinant Antibody (CBFYH-0262) (CAT#: CBMAB-H1135-FY)

Mouse Anti-GATM Recombinant Antibody (CBLG1-2624) (CAT#: CBMAB-G2331-LY)

Mouse Anti-GATM Recombinant Antibody (CBLG1-2625) (CAT#: CBMAB-G2332-LY)

Mouse Anti-GATM Recombinant Antibody (1C9) (CAT#: CBMAB-G2328-LY)

Mouse Anti-GATM Recombinant Antibody (1E3) (CAT#: CBMAB-G2329-LY)

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Request bulk or custom quote Second antibody and isotype control

Contact us

Tel: (USA)
(UK)
Fax:

Global Locations

Email:

Online Inquiry