Rabbit PYGL Polyclonal Antibody | anti-PYGL antibody

Rabbit Polyclonal to Human PYGL

Cat. Num. AAA240020

• Gene Names: PYGL; GSD6
• Reactivity: Dog, Gorilla, Human; Predicted Reactivity: Gibbon, Monkey (at least 90% immunogen sequence identity)
• Applications: Immunohistochemistry
• Purity: Immunoaffinity Purified
• Synonyms: PYGL; Polyclonal Antibody; Rabbit Polyclonal to Human PYGL; Anti-PYGL Antibody (Internal) IHC-plus; GPLL; Phosphorylase; glycogen liver; glycogen; liver; GSD6; Human PYGL; anti-PYGL antibody

• Host: Rabbit
• Reactivity: Dog, Gorilla, Human; Predicted Reactivity: Gibbon, Monkey (at least 90% immunogen sequence identity)
• Clonality: Polyclonal
• Specificity: Human PYGL. BLAST analysis of the peptide immunogen showed no homology with other human proteins.
• Purity/Purification: Immunoaffinity Purified
• Form/Format: PBS, 0.1% sodium azide.
• Concentration: 1 mg/ml (varies by lot)
• Sequence Length: 847

• Applicable Applications for anti-PYGL antibody: Immunohistochemistry (IHC - Paraffin)
• Application Notes: IHC-P (10 ug/ml); Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary anti...
• Target Species: Human
• Immunogen Description: Synthetic 10 amino acid peptide from internal region of human PYGL. Percent identity with other species by BLAST analysis: Human, Gorilla, Monkey, Dog (100%); Gibbon, Marmoset, Panda (90%); Mouse, Sheep, Hamster, Horse, Rabbit (80%).
• Immunogen Type: Synthetic peptide
• Immunogen: PYGL antibody was raised against synthetic 10 amino acid peptide from internal region of human PYGL. Percent identity with other species by BLAST analysis: Human, Gorilla, Monkey, Dog (100%); Gibbon, Marmoset, Panda (90%); Mouse, Sheep, Hamster, Horse, Rabbit (80%).
• Antigen Modification: Internal
• Preparation and Storage: Long term: -70 degree C; Short term: +4 degree C

Immunohistochemistry (IHC)

(Anti-PYGL antibody IHC of human liver. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval.)

NCBI and Uniprot Product Information

• NCBI GI #: 71037379
• NCBI GeneID: 5836
• NCBI Accession #: NP_002854.3
• NCBI GenBank Nucleotide #: NM_002863.4
• UniProt Accession #: P06737; O60567; O60752; O60913; Q501V9; Q641R5; Q96G82; A6NDQ4; B4DUB7; F5H816
• Molecular Weight: 93,134 Da
• NCBI Official Full Name: glycogen phosphorylase, liver form isoform 1
• NCBI Official Synonym Full Names: phosphorylase, glycogen, liver
• NCBI Official Symbol: PYGL
• NCBI Official Synonym Symbols: GSD6
• NCBI Protein Information: glycogen phosphorylase, liver form
• UniProt Protein Name: Glycogen phosphorylase, liver form
• Protein Family: Glycogen phosphorylase
• UniProt Gene Name: PYGL
• UniProt Entry Name: PYGL_HUMAN

NCBI Description

This gene encodes a homodimeric protein that catalyses the cleavage of alpha-1,4-glucosidic bonds to release glucose-1-phosphate from liver glycogen stores. This 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 genes that encode distinct 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, also known as 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. Alternative splicing results in multiple transcript variants encoding different isoforms.[provided by RefSeq, Feb 2011]

Uniprot Description

PYGL: Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties. Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6). A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected. Belongs to the glycogen phosphorylase family.

Protein type: Carbohydrate Metabolism - starch and sucrose; Transferase; EC 2.4.1.1

Chromosomal Location of Human Ortholog: 14q21-q22

Cellular Component: cytoplasm; plasma membrane; cytosol

Molecular Function: glycogen phosphorylase activity; protein homodimerization activity; bile acid binding; purine binding; drug binding; vitamin binding; ATP binding; pyridoxal phosphate binding; AMP binding; glucose binding

Biological Process: glycogen metabolic process; glycogen catabolic process; carbohydrate metabolic process; glucose metabolic process; 5-phosphoribose 1-diphosphate biosynthetic process; pathogenesis; glucose homeostasis

Disease: Glycogen Storage Disease Vi