Recombinant Human AGL Protein, Myc/DDK-tagged, C13 and N15-labeled

• Cat.No.: AGL-2755H
• Product Overview: AGL MS Standard C13 and N15-labeled recombinant protein (NP_000633) with a C-terminal MYC/DDK tag, was expressed in HEK293 cells.

Specification

• Description: This gene encodes the glycogen debrancher enzyme which is involved in glycogen degradation. This enzyme has two independent catalytic activities which occur at different sites on the protein: a 4-alpha-glucotransferase activity and a amylo-1,6-glucosidase activity. Mutations in this gene are associated with glycogen storage disease although a wide range of enzymatic and clinical variability occurs which may be due to tissue-specific alternative splicing. Alternatively spliced transcripts encoding different isoforms have been described.
• Source: HEK293
• Species: Human
• Tag: Myc/DDK
• Molecular Mass: 174.6 kDa
• AA Sequence: MGHSKQIRILLLNEMEKLEKTLFRL EQGYELQFRLGPTLQGKAVTVYTNY PFPGETFNREKFRSLDWENPTERED DSDKYCKLNLQQSGSFQYYFLQGNE KSGGGYIVVDPILRVGADNHVLPLD CVTLQTFLAKCLGPFDEWESRLRVA KESGYNMIHFTPLQTLGLSRSCYSL ANQLELNPDFSRPNRKYTWNDVGQL VEKLKKEWNVICITDVVYNHTAANS KWIQEHPECAYNLVNSPHLKPAWVL DRALWRFSCDVAEGKYKEKGIPALI ENDHHMNSIRKIIWEDIFPKLKLWE FFQVDVNKAVEQFRRLLTQENRRVT KSDPNQHLTIIQDPEYRRFGCTVDM NIALTTFIPHDKGPAAIEECCNWFH KRMEELNSEKHRLINYHQEQAVNCL LGNVFYERLAGHGPKLGPVTRKHPL VTRYFTFPFEEIDFSMEESMIHLPN KACFLMAHNGWVMGDDPLRNFAEPG SEVYLRRELICWGDSVKLRYGNKPE DCPYLWAHMKKYTEITATYFQGVRL DNCHSTPLHVAEYMLDAARNLQPNL YVVAELFTGSEDLDNVFVTRLGISS LIREAMSAYNSHEEGRLVYRYGGEP VGSFVQPCLRPLMPAIAHALFMDIT HDNECPIVHRSAYDALPSTTIVSMA CCASGSTRGYDELVPHQISVVSEER FYTKWNPEALPSNTGEVNFQSGIIA ARCAISKLHQELGAKGFIQVYVDQV DEDIVAVTRHSPSIHQSVVAVSRTA FRNPKTSFYSKEVPQMCIPGKIEEV VLEARTIERNTKPYRKDENSINGTP DITVEIREHIQLNESKIVKQAGVAT KGPNEYIQEIEFENLSPGSVIIFRV SLDPHAQVAVGILRNHLTQFSPHFK SGSLAVDNADPILKIPFASLASRLT LAELNQILYRCESEEKEDGGGCYDI PNWSALKYAGLQGLMSVLAEIRPKN DLGHPFCNNLRSGDWMIDYVSNRLI SRSGTIAEVGKWLQAMFFYLKQIPR YLIPCYFDAILIGAYTTLLDTAWKQ MSSFVQNGSTFVKHLSLGSVQLCGV GKFPSLPILSPALMDVPYRLNEITK EKEQCCVSLAAGLPHFSSGIFRCWG RDTFIALRGILLITGRYVEARNIIL AFAGTLRHGLIPNLLGEGIYARYNC RDAVWWWLQCIQDYCKMVPNGLDIL KCPVSRMYPTDDSAPLPAGTLDQPL FEVIQEAMQKHMQGIQFRERNAGPQ IDRNMKDEGFNITAGVDEETGFVYG GNRFNCGTWMDKMGESDRARNRGIP ATPRDGSAVEIVGLSKSAVRWLLEL SKKNIFPYHEVTVKRHGKAIKVSYD EWNRKIQDNFEKLFHVSEDPSDLNE KHPNLVHKRGIYKDSYGASSPWCDY QLRPNFTIAMVVAPELFTTEKAWKA LEIAEKKLLGPLGMKTLDPDDMVYC GIYDNALDNDNYNLAKGFNYHQGPE WLWPIGYFLRAKLYFSRLMGPETTA KTIVLVKNVLSRHYVHLERSPWKGL PELTNENAQYCPFSCETQAWSIATI LETLYDLTRTRPLEQKLISEEDLAA NDILDYKDDDDKV
• Purity: > 80% as determined by SDS-PAGE and Coomassie blue staining
• Stability: Stable for 3 months from receipt of products under proper storage and handling conditions.
• Storage: Store at -80 centigrade. Avoid repeated freeze-thaw cycles.
• Concentration: 50 μg/mL as determined by BCA
• Storage Buffer: 100 mM glycine, 25 mM Tris-HCl, pH 7.3.

Gene Information

• Gene Name: AGL amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase [ Homo sapiens (human) ]
• Official Symbol: AGL
• Synonyms: AGL; amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase; amylo 1, 6 glucosidase, 4 alpha glucanotransferase; glycogen debranching enzyme; glycogen storage disease type III; glycogen debrancher; amylo-1, 6-glucosidase, 4-alpha-glucanotransferase; GDE
• Gene ID: 178
• mRNA Refseq: NM_000642
• Protein Refseq: NP_000633
• MIM: 610860
• UniProt ID: P35573

Reviews

05/17/2019

 Notable improvements in cell proliferation were attributed to the protein product’s efficacy.

11/30/2018

 We observed a significant enhancement in enzyme activity using the protein product.

09/11/2018

 The protein product’s compatibility with various assays broadened the scope of our investigations.

08/25/2017

 Reliable protein-protein interaction studies were enabled by the protein product’s pure composition.

08/20/2017

 Minimal background noise in our assays indicated the protein product’s quality.

Q&As

Can AGL mutations lead to hypoglycemia?

Yes, AGL mutations can cause low blood sugar levels due to impaired glucose release.

Can AGL mutations lead to cardiomyopathy?

AGL mutations might contribute to cardiomyopathy due to glycogen accumulation.

Can AGL mutations cause muscle weakness?

Yes, AGL mutations can lead to muscle weakness due to impaired glycogen breakdown.

Is AGL's role conserved across species?

Research explores whether AGL's function is conserved in different organisms.

How does AGL influence cellular glycogen stores?

AGL activity ensures proper glycogen breakdown, maintaining cellular glycogen levels.

Is AGL research ongoing?

Yes, ongoing research uncovers AGL's roles in metabolism and related diseases.

How does AGL contribute to glycogen structure?

AGL's enzymatic activity alters glycogen structure by removing branches.

Can AGL be a target for therapeutic interventions?

AGL's role in glycogen metabolism makes it a potential therapeutic target.

How does AGL influence glycogen synthesis?

AGL's enzymatic role is in breaking down glycogen, not synthesizing it.

How does AGL dysfunction impact different tissues?

AGL dysfunction can affect various tissues due to glycogen storage disruptions.

How does AGL deficiency impact muscle cells?

AGL deficiency affects muscle cells due to impaired glycogen breakdown.

What's the significance of AGL research?

Research on AGL provides insights into glycogen metabolism and related diseases.

Is AGL activity regulated by hormones?

Yes, hormones regulate AGL activity to maintain glucose levels.

Is AGL involved in heart health?

Yes, AGL's role in glycogen breakdown can impact heart function and health.

How does AGL contribute to metabolic homeostasis?

AGL's role in glycogen breakdown helps maintain glucose homeostasis.

Is AGL deficiency inherited?

Yes, AGL deficiency is usually inherited in an autosomal recessive manner.

How does AGL deficiency impact energy availability?

AGL deficiency hinders glucose release from glycogen, affecting energy supply.

Can AGL mutations affect exercise tolerance?

Yes, AGL mutations can impact exercise tolerance due to limited energy supply.

Can AGL mutations lead to multi-organ involvement?

Yes, AGL mutations can impact multiple organs due to glycogen storage issues.

Can AGL mutations affect brain function?

AGL mutations can impact brain health due to glycogen storage issues in certain tissues.