GATM Antibody

Datasheet

Code	CSB-PA009284ESR1HU
Size	US$166
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Image	Western blot All lanes: Glycine amidinotransferase, mitochondrial antibody at 2μg/ml + Mouse kidney tissue Secondary Goat polyclonal to rabbit IgG at 1/10000 dilution Predicted band size: 49, 45 kDa Observed band size: 49 kDa Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA009284ESR1HU at dilution of 1:100 Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA009284ESR1HU at dilution of 1:100
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Product Details
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) GATM Polyclonal antibody

Uniprot No.

P50440

Target Names

GATM

Alternative Names

AGAT antibody; AT antibody; GATM antibody; GATM_HUMAN antibody; Glycine amidinotransferase antibody; Glycine amidinotransferase, mitochondrial antibody; L-arginine:glycine amidinotransferase antibody; mitochondrial antibody; Transamidinase antibody

Raised in

Rabbit

Species Reactivity

Human, Mouse

Immunogen

Recombinant Human Glycine amidinotransferase, mitochondrial protein (124-423AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Polyclonal

Isotype

IgG

Purification Method

Antigen Affinity Purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Form

Liquid

Tested Applications

ELISA, WB, IHC

Recommended Dilution

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:20-1:200

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

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Target Background

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.

Gene References into Functions

Measurements of creatine and guanidinoacetate in plasma are recommended for the diagnosis of AGAT and GAMT deficiency.Definitive confirmation of the diagnosis requires DNA sequencing of the appropriate gene and (if molecular analysis is ambiguous) measurement of AGAT or GAMT enzyme activity or of CRTR-mediated transport . PMID: 28055022
Results show the functional characterization of rare missense variants in GATM which cause GATM deficiency. Seven of them report 0% of wild-type GATM activity indicating a putative pathogenicity. PMID: 27233232
AGAT deficiency is a treatable intellectual disability. PMID: 26490222
Meta-analysis yielded a marginal, but null, association of GATM rs9806699 with statin-induced myopathy. PMID: 25863251
Genome-wide association reveals that plasma homoarginine is strongly associated with single nucleotide polymorphisms in the AGAT gene. PMID: 24004504
promiscuous activity of AGAT, a key enzyme in creatine synthesis, plays a pivotal role in homoarginine synthesis PMID: 23010440
GATM sequencing revealed a homozygous single nucleotide insertion 1111_1112insA, producing a frame-shift at Met-371 and premature termination at codon 376. PMID: 20682460
AGAT mRNA expression was significantly elevated in all heart failure patients and returned to normal levels after recovery, suggesting a specific response to heart failure involving elevated local creatine synthesis. PMID: 16820567

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Involvement in disease

Cerebral creatine deficiency syndrome 3 (CCDS3)

Subcellular Location

[Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side. Note=Probably attached to the outer side of the inner membrane.; [Isoform 2]: Cytoplasm.

Protein Families

Amidinotransferase family

Tissue Specificity

Expressed in brain, heart, kidney, liver, lung, salivary gland and skeletal muscle tissue, with the highest expression in kidney. Biallelically expressed in placenta and fetal tissues.

Database Links

HGNC: 4175

OMIM: 602360

KEGG: hsa:2628

STRING: 9606.ENSP00000379895

UniGene: Hs.560354

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Antibody FAQs What Is an Antibody? How to Choose an Antibody for Scientific Research? The difference between primary antibody and secondary antibod The Meaning of Irregular Antibody Screening Tag Antibodies, Your Good Partner in Protein Research How to Choose the Loading Control Antibodies?

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