PHKA2 (phosphorylase kinase regulatory subunit alpha 2)

2003-11-01  

Identity

HGNC
LOCATION
Xp22.13
LOCUSID
ALIAS
GSD9A,PHK,PYK,PYKL,XLG,XLG2
FUSION GENES

Other Information

Locus ID:

NCBI: 5256
MIM: 300798
HGNC: 8926
Ensembl: ENSG00000044446

Variants:

dbSNP: 5256
ClinVar: 5256
TCGA: ENSG00000044446
COSMIC: PHKA2

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000044446ENST00000379942P46019

Expression (GTEx)

0
5
10
15
20
25
30
35

Pathways

PathwaySourceExternal ID
Calcium signaling pathwayKEGGko04020
Insulin signaling pathwayKEGGko04910
Calcium signaling pathwayKEGGhsa04020
Insulin signaling pathwayKEGGhsa04910
Glucagon signaling pathwayKEGGhsa04922
Glucagon signaling pathwayKEGGko04922
MetabolismREACTOMER-HSA-1430728
Metabolism of carbohydratesREACTOMER-HSA-71387
Glucose metabolismREACTOMER-HSA-70326
Glycogen breakdown (glycogenolysis)REACTOMER-HSA-70221

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
341178282021PHKA2 variants expand the phenotype of phosphorylase B kinase deficiency to include patients with ketotic hypoglycemia only.3
341178282021PHKA2 variants expand the phenotype of phosphorylase B kinase deficiency to include patients with ketotic hypoglycemia only.3
323876372020A novel frameshift PHKA2 mutation in a family with glycogen storage disease type IXa: A first report in Vietnam and review of literature.2
333177992020Benign or not benign? Deep phenotyping of liver Glycogen Storage Disease IX.13
323876372020A novel frameshift PHKA2 mutation in a family with glycogen storage disease type IXa: A first report in Vietnam and review of literature.2
333177992020Benign or not benign? Deep phenotyping of liver Glycogen Storage Disease IX.13
309259022019A novel PHKA2 mutation in a Chinese child with glycogen storage disease type IXa: a case report and literature review.7
309259022019A novel PHKA2 mutation in a Chinese child with glycogen storage disease type IXa: a case report and literature review.7
286274412017Clinical and genetic characteristics of 17 Chinese patients with glycogen storage disease type IXa.10
286274412017Clinical and genetic characteristics of 17 Chinese patients with glycogen storage disease type IXa.10
271033792016PHKA2 mutation spectrum in Korean patients with glycogen storage disease type IX: prevalence of deletion mutations.12
271033792016PHKA2 mutation spectrum in Korean patients with glycogen storage disease type IX: prevalence of deletion mutations.12
218572512012Glycogen storage disease type IX: novel PHKA2 missense mutation and cirrhosis.14
218572512012Glycogen storage disease type IX: novel PHKA2 missense mutation and cirrhosis.14
211312182011Novel mutations in PHKA2 gene in glycogen storage disease type IX patients from Hong Kong, China.7

Citation

Dessen P

PHKA2 (phosphorylase kinase regulatory subunit alpha 2)

Atlas Genet Cytogenet Oncol Haematol. 2003-11-01

Online version: http://atlasgeneticsoncology.org/gene/41705/phka2