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Glycogen Phosphorylase, Liver (PYGL)

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GPLL; GPBB; Hers Disease; Glycogen Storage Disease Type VI

Glycogen Phosphorylase, Liver (PYGL)
CCC A849
HGNC 9725
MGI 97829
PubMed 10980448
RGD 620687
UniProt P06737

PYGL is a homodimeric protein switches from inactive phosphorylase B to active phosphorylase A by phosphorylation of serine residue 15.

Activity of this enzyme is further regulated by multiple allosteric effectors and hormonal controls. Humans have three glycogen phosphorylase isozymes that are primarily expressed in liver, brain and muscle, respectively. The liver isozyme serves the glycemic demands of the body in general while the brain and muscle isozymes supply just those tissues. In glycogen storage disease type VI, or Hers disease, mutations in liver glycogen phosphorylase inhibit the conversion of glycogen to glucose and results in moderate hypoglycemia, mild ketosis, growth retardation and hepatomegaly.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA849Hu01 Recombinant Glycogen Phosphorylase, Liver (PYGL) Positive Control; Immunogen; SDS-PAGE; WB.
RPA849Hu02 Recombinant Glycogen Phosphorylase, Liver (PYGL) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA849Hu01 Polyclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB; IHC; ICC; IP.
MAA849Hu22 Monoclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB; IHC; ICC; IP.
PAA849Hu02 Polyclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB,IHC
LAA849Hu71 Biotin-Linked Polyclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB; IHC; ICC.
Assay Kits SEA849Hu ELISA Kit for Glycogen Phosphorylase, Liver (PYGL) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA849Mu01 Recombinant Glycogen Phosphorylase, Liver (PYGL) Positive Control; Immunogen; SDS-PAGE; WB.
RPA849Mu02 Recombinant Glycogen Phosphorylase, Liver (PYGL) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA849Mu01 Polyclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB; IHC; ICC; IP.
PAA849Mu02 Polyclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB; IHC; ICC; IP.
Assay Kits SEA849Mu ELISA Kit for Glycogen Phosphorylase, Liver (PYGL) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA849Ra01 Recombinant Glycogen Phosphorylase, Liver (PYGL) Positive Control; Immunogen; SDS-PAGE; WB.
RPA849Ra02 Recombinant Glycogen Phosphorylase, Liver (PYGL) Positive Control; Immunogen; SDS-PAGE; WB.
RPA849Ra03 Recombinant Glycogen Phosphorylase, Liver (PYGL) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA849Ra01 Polyclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB,IHC
PAA849Ra02 Polyclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB; IHC; ICC; IP.
PAA849Ra03 Polyclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB; IHC; ICC; IP.
LAA849Ra71 Biotin-Linked Polyclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB; IHC; ICC.
MAA849Ra21 Monoclonal Antibody to Glycogen Phosphorylase, Liver (PYGL) WB; IHC; ICC; IP.
Assay Kits SEA849Ra ELISA Kit for Glycogen Phosphorylase, Liver (PYGL) Enzyme-linked immunosorbent assay for Antigen Detection.
  1. "Sequence analysis of the cDNA encoding human liver glycogen phosphorylase reveals tissue-specific codon usage."Proc. Natl. Acad. Sci. U.S.A. 83:8132-8136(1986) [PubMed] [Europe PMC] [Abstract]
  2. "Identification of a mutation in liver glycogen phosphorylase in glycogen storage disease type VI."Hum. Mol. Genet. 7:865-870(1998) [PubMed] [Europe PMC] [Abstract]
  3. "Mutations in the liver glycogen phosphorylase gene 'PYGL' underlying glycogenosis type VI."Am. J. Hum. Genet. 62:785-791(1998) [PubMed] [Europe PMC] [Abstract]
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  5. "The DNA sequence and analysis of human chromosome 14." Nature 421:601-607(2003) [PubMed] [Europe PMC] [Abstract]
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  7. "McArdle's & Hers' diseases: glycogen phosphorylase transcriptional expression in human tissues."J. Neurogenet. 4:293-308(1987) [PubMed] [Europe PMC] [Abstract]
  8. "Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach."Anal. Chem. 81:4493-4501(2009) [PubMed] [Europe PMC] [Abstract]
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  10. "N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB."Proc. Natl. Acad. Sci. U.S.A. 109:12449-12454(2012) [PubMed] [Europe PMC] [Abstract]
  11. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
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  13. "Activation of human liver glycogen phosphorylase by alteration of the secondary structure and packing of the catalytic core."Mol. Cell 6:139-148(2000) [PubMed] [Europe PMC] [Abstract]
  14. "Structure-activity analysis of the purine binding site of human liver glycogen phosphorylase."Chem. Biol. 9:915-924(2002) [PubMed] [Europe PMC] [Abstract]