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Phosphorylase kinase, alpha 2

Phosphorylase kinase is a polymer of 16 subunits, four each of alpha, beta, gamma and delta. The alpha subunit includes the skeletal muscle and hepatic isoforms, and the hepatic isoform is encoded by this gene. The beta subunit is the same in both the muscle and hepatic isoforms, and encoded by one gene. The gamma subunit also includes the skeletal muscle and hepatic isoforms, which are encoded by two different genes. The delta subunit is a calmodulin and can be encoded by three different genes. The gamma subunits contain the active site of the enzyme, whereas the alpha and beta subunits have regulatory functions controlled by phosphorylation. The delta subunit mediates the dependence of the enzyme on calcium concentration. Mutations in this gene cause glycogen storage disease type 9A, also known as X-linked liver glycogenosis. Alternatively spliced transcript variants have been reported, but the full-length nature of these variants has not been determined.[provided by RefSeq, Feb 2010] (from NCBI)
Top mentioned proteins: CAN, ACID, V1a, HAD, Src
Papers on PHKA2
Effects of phosphoenolpyruvate carboxylase desensitization on glutamic acid production in Corynebacterium glutamicum ATCC 13032.
Yokota et al., Sapporo, Japan. In J Biosci Bioeng, Feb 2016
Another mutant, strain DR1, having both desensitized PEPC and PYK-gene deleted mutations, was constructed in a similar manner using strain D1 with a PYK-gene deleted mutation as the parent.
The network of surface-displayed glycolytic enzymes in Mycoplasma pneumoniae and their interactions with human plasminogen.
Dumke et al., Dresden, Germany. In Infect Immun, Jan 2016
Eight glycolytic enzymes, pyruvate dehydrogenase A-C (PdhA-C), glyceraldehyde-3-phosphate dehydrogenase (GapA), lactate dehydrogenase (Ldh), phosphoglycerate mutase (Pgm), pyruvate kinase (Pyk) and transketolase (Tkt), were confirmed as surface-expressed and all are able to interact with plasminogen.
Antitumor effect of metformin on cholangiocarcinoma: In vitro and in vivo studies.
Masaki et al., Japan. In Oncol Rep, Dec 2015
In addition, it reduced the phosphorylation of Axl, EphA10, ALK and PYK, as well as tumor proliferation in athymic nude mice with xenograft tumors.
Src kinase as a mediator of convergent molecular abnormalities leading to NMDAR hypoactivity in schizophrenia.
Hahn et al., Philadelphia, United States. In Mol Psychiatry, Sep 2015
At the postreceptor level, however, we found striking reductions in the protein kinase C, Pyk 2 and Src kinase activity that in tandem can decrease GluN2 activation.
Extra-Large G Proteins Expand the Repertoire of Subunits in Arabidopsis Heterotrimeric G Protein Signaling.
Assmann et al., United States. In Plant Physiol, Sep 2015
In addition to GPA1, the sole canonical Arabidopsis (Arabidopsis thaliana) Gα subunit, Arabidopsis has three related proteins: the extra-large GTP-binding proteins XLG1, XLG2, and XLG3.
Clinical, Biochemical, and Genetic Characterization of Glycogen Storage Type IX in a Child with Asymptomatic Hepatomegaly.
Kim et al., Seoul, South Korea. In Pediatr Gastroenterol Hepatol Nutr, Jun 2015
Glycogen storage disease type IX (GSD IX) is caused by a defect in phosphorylase b kinase (PhK) that results from mutations in the PHKA2, PHKB, and PHKG2 genes.
Distinguishing the interactions in the fructose 1,6-bisphosphate binding site of human liver pyruvate kinase that contribute to allostery.
Fenton et al., Kansas City, United States. In Biochemistry, Mar 2015
The antihyperglycemic target, human liver pyruvate kinase (hL-PYK), binds its allosteric activator, fructose 1,6-bisphosphate (Fru-1,6-BP), such that the 1'-phosphate interacts with side chains of Arg501 and Trp494 and the 6'-phosphate interacts with Thr444, Thr446, Ser449 (i.e., the 444-449 loop), and Ser531.
Functional role of pyruvate kinase from Lactobacillus bulgaricus in acid tolerance and identification of its transcription factor by bacterial one-hybrid.
Hao et al., Beijing, China. In Sci Rep, 2014
In this study, pyk gene encoding pyruvate kinase was over-expressed in heterologous host Lactococcus lactis NZ9000, and SDS-PAGE analysis revealed the successful expression of this gene in NZ9000.
Identification of RNAIII-binding proteins in Staphylococcus aureus using tethered RNAs and streptavidin aptamers based pull-down assay.
Sun et al., Hefei, China. In Bmc Microbiol, 2014
The binding ability of the recombinant proteins with RNAIII was further analyzed by electrophoresis mobility shift assay, and the result indicates that proteins CshA, RNase J2, Era, Hu, WalR, Pyk, and FtsZ can bind to RNAIII.
Structures of pyruvate kinases display evolutionarily divergent allosteric strategies.
Walkinshaw et al., Edinburgh, United Kingdom. In R Soc Open Sci, 2014
The transition between the inactive T-state (apoenzyme) and active R-state (effector bound enzyme) of Trypanosoma cruzi pyruvate kinase (PYK) is accompanied by a symmetrical 8° rigid body rocking motion of the A- and C-domain cores in each of the four subunits, coupled with the formation of additional salt bridges across two of the four subunit interfaces.
Novel PHKG2 mutation causing GSD IX with prominent liver disease: report of three cases and review of literature.
Al-Owain et al., Kuwait, Kuwait. In Eur J Pediatr, 2014
Glycogen storage disease type IX (GSD IX) is a common form of glycogenosis due to mutations in PHKA1, PHKA2, or PHKB and PHKG2 genes resulting in the deficiency of phosphorylase kinase.
Glycogen storage disease type IX: novel PHKA2 missense mutation and cirrhosis.
Bali et al., Amarillo, United States. In J Pediatr Gastroenterol Nutr, 2012
The present case also represents the first known reported case of liver PhK deficiency with an PHKA2 mutation and liver cirrhosis.
Common mutation in the PHKA2 gene with variable phenotype in patients with liver phosphorylase b kinase deficiency.
Morava et al., Nijmegen, Netherlands. In Mol Genet Metab, 2011
We found that the missense mutation p.Pro1205Leu in the PHKA2 gene is a common cause of hepatic phosphorylase-kinase deficiency in Dutch patients, suggesting a founder-effect
Pyruvate kinase triggers a metabolic feedback loop that controls redox metabolism in respiring cells.
Ralser et al., Berlin, Germany. In Cell Metab, 2011
Studying its regulator pyruvate kinase (PYK) in yeast, we discovered that central metabolism is self-adapting to synchronize redox metabolism when respiration is activated.
Phosphorylase Kinase Deficiency
Bali et al., Seattle, United States. In Unknown Journal, 2011
Mutations in PHKA1, encoding subunit α, cause the rare X-linked disorder muscle PhK deficiency; mutations in PHKA2, also encoding subunit α, cause the most common form, liver PhK deficiency (X-linked liver glycogenosis); mutations in PHKB, encoding subunit β, cause autosomal recessive PhK deficiency in both liver and muscle; and mutations in PHKG2, encoding subunit γ, cause autosomal recessive liver PhK deficiency.
Novel mutations in PHKA2 gene in glycogen storage disease type IX patients from Hong Kong, China.
Tsui et al., Hong Kong, Hong Kong. In Mol Genet Metab, 2011
two novel mutations found in two GSD type IX patients with different residual enzyme activities
HIF-1alpha modulates energy metabolism in cancer cells by inducing over-expression of specific glycolytic isoforms.
Moreno-Sánchez et al., Mexico. In Mini Rev Med Chem, 2009
In cancer cells, HIF-1alpha induces over-expression and increased activity of several glycolytic protein isoforms that differ from those found in non-malignant cells, including transporters (GLUT1, GLUT3) and enzymes (HKI, HKII, PFK-L, ALD-A, ALD-C, PGK1, ENO-alpha, PYK-M2, LDH-A, PFKFB-3).
3D mapping of glycogenosis-causing mutations in the large regulatory alpha subunit of phosphorylase kinase.
Callebaut et al., Paris, France. In Biochim Biophys Acta, 2008
Results show that phosphorylase kinase (PhK) alpha subunit missense mutations or small in-frame deletions/insertions may have a direct impact on the PhK alpha functions.
Identification of Alu-mediated, large deletion-spanning introns 19-26 in PHKA2 in a patient with X-linked liver glycogenosis (hepatic phosphorylase kinase deficiency).
Kondo et al., Gifu, Japan. In Mol Genet Metab, 2007
Alu-mediated, large deletion-spanning introns 19-26 in PHKA2 is associated with X-linked liver glycogenosis
Absence of marginal zone B cells in Pyk-2-deficient mice defines their role in the humoral response.
Ravetch et al., New York City, United States. In Nat Immunol, 2000
Here we demonstrate that a deficiency of the tyrosine kinase (Pyk-2) results in a cell autonomous defect of MZB cell production.
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