Glycogen Storage Disease Type VII

An autosomal recessive glycogen storage disease in which there is deficient expression of 6-phosphofructose 1-kinase in muscle (PHOSPHOFRUCTOKINASE-1, MUSCLE TYPE) resulting in abnormal deposition of glycogen in muscle tissue. These patients have severe congenital muscular dystrophy and are exercise intolerant.

Synonym
Glycogen Storage Disease Type VII

Timeframe	Studies, This Condition (%)	All Conditions %
pre-1990	12 (33.33)	23.3326
1990's	14 (38.89)	12.5806
2000's	8 (22.22)	18.1394
2010's	2 (5.56)	28.8240
2020's	0 (0.00)	9.53

Drug	Relationship Strength	Studies	Trials
2,3-diphosphoglycerate	low	2	0
ammonium hydroxide	medium	5	2
carnitine	low	2	0
lactic acid	medium	3	1
pyruvic acid	low	1	0
uric acid	low	5	0
alanine	low	1	0
adenosine diphosphate	low	1	0
adenosine monophosphate	low	2	0
asparagine	low	1	0
tryptophan	low	1	0
fructose-6-phosphate	low	1	0
glucose-1,6-bisphosphate	low	1	0
p-methoxy-n-methylphenethylamine	low	1	0
fructose 2,6-diphosphate	low	2	0
glycogen	low	15	0
bilirubin	low	1	0
cysteine	low	1	0
phosphocreatine	low	2	0
hypoxanthine	medium	4	1
inosinic acid	low	1	0
inosine	medium	5	1

Protein Targets (266)

Protein	Potency Measurements	Inhibition Measurements	Activation Measurements	Drugs
Chain A, Heat Shock Protein 90	0	0	1	1
Chain A, GLUTAMINE PHOSPHORIBOSYLPYROPHOSPHATE AMIDOTRANSFERASE	0	0	1	1
Chain B, GLUTAMINE PHOSPHORIBOSYLPYROPHOSPHATE AMIDOTRANSFERASE	0	0	1	1
Chain A, EOSINOPHIL-DERIVED NEUROTOXIN	0	1	0	1
Chain A, EOSINOPHIL-DERIVED NEUROTOXIN	0	1	0	1
Chain A, EOSINOPHIL-DERIVED NEUROTOXIN	0	1	0	1
Chain A, Myosin Ie Heavy Chain	0	0	1	1
Chain A, Preprotein translocase secA	0	0	1	1
Chain A, Ribonuclease pancreatic	0	1	0	1
Chain A, Ribonuclease pancreatic	0	1	0	1
Chain A, Ribonuclease pancreatic	0	1	0	1
Chain A, Ribonuclease pancreatic	0	1	0	1
Chain A, Ribonuclease pancreatic	0	1	0	1
Chain A, Phosphoribosylformylglycinamidine synthase	0	1	0	1
Chain A, nucleoside diphosphate kinase A	0	0	1	1
Chain B, nucleoside diphosphate kinase A	0	0	1	1
Chain D, DNA polymerase III subunit gamma	0	0	1	1
Chain D, DNA polymerase III subunit gamma	0	0	1	1
Chain A [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 3	0	0	1	1
Chain A [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 3	0	0	1	1
Chain A, Kinesin-like protein KIF11	0	1	0	1
regulator of G-protein signaling 4	2	0	0	2
HPr kinase/phosphorylase	0	0	1	1
Mitogen-activated protein kinase kinase kinase 7	0	0	2	2
ATP-dependent molecular chaperone HSP82	0	0	0	1
Heat shock protein HSP 90-alpha	0	1	1	3
Heat shock protein HSP 90-beta	0	1	1	3
2-dehydropantoate 2-reductase	0	2	2	4
Heat shock 70 kDa protein 1A	0	1	1	2
Endoplasmic reticulum chaperone BiP	0	0	1	1
Heat shock cognate 71 kDa protein	0	0	1	1
Pyruvate kinase PKM	0	0	0	1
Pyruvate kinase PKLR	0	0	0	1
Endoplasmin	0	0	0	1
Heat shock cognate 71 kDa protein	0	0	1	1
5'-nucleotidase	0	1	0	1
Mu-type opioid receptor	0	0	1	1
Delta-type opioid receptor	0	0	1	1
Kappa-type opioid receptor	0	0	1	1
Endoplasmin	0	1	1	2
P2Y purinoceptor 2	0	0	2	2
Phosphatidylinositol 4-kinase alpha	0	2	0	2
P2X purinoceptor 1	0	0	1	1
P2Y purinoceptor 1	0	0	2	2
P2Y purinoceptor 1	0	0	2	2
P2X purinoceptor 1	0	0	1	1
P2X purinoceptor 4	0	0	1	1
P2X purinoceptor 5	0	0	1	1
P2X purinoceptor 6	0	0	1	1
P2X purinoceptor 3	0	0	1	1
Inositol monophosphatase 1	2	0	0	2
Heat shock protein 75 kDa, mitochondrial	0	1	0	1
P2Y purinoceptor 6	0	0	1	1
Phosphatidylinositol 4-kinase type 2-beta	0	2	0	2
P2Y purinoceptor 11	0	0	2	2
Phosphatidylinositol 4-kinase type 2-alpha	0	2	0	2
P2Y purinoceptor 12	0	0	1	1
Sensor protein kinase WalK	0	1	0	1
Phosphatidylinositol 4-kinase beta	0	2	0	2
P2X purinoceptor 2	0	0	1	1
Chain A, Glycogen Phosphorylase B	0	0	1	1
Chain B, Glycogen Phosphorylase B	0	0	1	1
Chain A, ADP-dependent glucokinase	0	1	0	1
Chain A, Ribonuclease pancreatic	0	2	0	2
Chain A, Ribonuclease pancreatic	0	2	0	2
Chain A, phosphodiesterase-nucleotide pyrophosphatase	0	1	0	1
Chain A, GLYCOGEN PHOSPHORYLASE B	0	0	1	1
Chain B, GLYCOGEN PHOSPHORYLASE B	0	0	1	1
Chain A, GLYCOGEN PHOSPHORYLASE B	0	0	1	1
RAR-related orphan receptor gamma	2	0	0	2
AR protein	4	0	0	4
glucocorticoid receptor [Homo sapiens]	2	0	0	2
estrogen-related nuclear receptor alpha	4	0	0	4
euchromatic histone-lysine N-methyltransferase 2	2	0	0	2
v-jun sarcoma virus 17 oncogene homolog (avian)	3	0	0	3
2'-deoxynucleoside 5'-phosphate N-hydrolase 1	0	1	0	1
2'-deoxynucleoside 5'-phosphate N-hydrolase 1	0	1	0	1
5'-AMP-activated protein kinase subunit beta-2	0	0	1	1
Transient receptor potential cation channel subfamily M member 2	0	1	0	1
Alcohol dehydrogenase E chain	0	1	0	1
Alcohol dehydrogenase S chain	0	1	0	1
L-lactate dehydrogenase A chain	0	1	1	2
Adenylate kinase isoenzyme 1	0	0	0	1
Fructose-1,6-bisphosphatase 1	0	1	0	1
Alkaline phosphatase, tissue-nonspecific isozyme	0	1	0	2
Fructose-1,6-bisphosphatase 1	0	1	0	1
Inosine-5'-monophosphate dehydrogenase	0	1	0	2
Cytochrome P450 2C9	0	1	0	1
Proto-oncogene tyrosine-protein kinase Src	0	1	0	1
Amine oxidase [flavin-containing] B	0	1	0	1
5'-nucleotidase	0	0	0	1
Choline O-acetyltransferase	0	1	0	1
Adenylate kinase 2, mitochondrial	0	1	0	2
GTP:AMP phosphotransferase AK3, mitochondrial	0	0	0	1
Adenosine receptor A1	0	0	1	1
Adenylate kinase isoenzyme 1	0	1	0	2
5'-AMP-activated protein kinase subunit gamma-1	0	0	2	2
5'-AMP-activated protein kinase catalytic subunit alpha-2	0	0	1	1
Adenylate kinase 2, mitochondrial	0	0	0	1
Glycine--tRNA ligase	0	1	0	1
5'-AMP-activated protein kinase catalytic subunit alpha-1	0	0	2	2
Adenosine deaminase-like protein	0	0	0	1
Protease	0	1	1	2
Histamine H3 receptor	0	1	0	1
5'-AMP-activated protein kinase subunit gamma-3	0	0	1	1
5'-AMP-activated protein kinase subunit gamma-2	0	0	1	1
5'-AMP-activated protein kinase subunit beta-1	0	0	2	2
ATP-dependent phosphofructokinase	2	0	0	2
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE	1	0	0	1
Chain A, TYROSYL-DNA PHOSPHODIESTERASE	2	0	0	2
Carbonic anhydrase 1	0	0	0	3
Carbonic anhydrase 2	0	0	0	3
Alanine racemase, biosynthetic	0	0	0	1
5-hydroxytryptamine receptor 1D	0	0	0	1
Glutamate receptor ionotropic, NMDA 1	0	1	0	2
Proton-coupled amino acid transporter 1	0	1	0	1
Adenosine deaminase	0	0	0	1
Carbonic anhydrase 4	0	0	0	1
Neutral amino acid transporter A	0	1	0	1
Neutral amino acid transporter B(0)	0	1	0	1
Carbonic anhydrase-like protein, putative	0	0	0	2
Amino acid transporter	0	1	0	1
Chain A, Putative fructose-1,6-bisphosphate aldolase	2	0	0	2
Chain A, HADH2 protein	1	0	0	1
Chain B, HADH2 protein	1	0	0	1
Chain A, 2-oxoglutarate Oxygenase	2	0	0	2
Chain A, Cruzipain	1	0	0	1
phosphopantetheinyl transferase	3	0	0	3
USP1 protein, partial	1	0	0	1
Microtubule-associated protein tau	2	0	0	2
aldehyde dehydrogenase 1 family, member A1	2	0	0	2
vitamin D3 receptor isoform VDRA	1	0	0	1
DNA polymerase kappa isoform 1	1	0	0	1
caspase-1 isoform alpha precursor	1	0	0	1
lamin isoform A-delta10	2	0	0	2
Caspase-7	1	0	0	1
Solute carrier organic anion transporter family member 1B3	0	2	0	3
Solute carrier organic anion transporter family member 1B1	0	2	0	3
Solute carrier family 22 member 5	0	0	0	1
Solute carrier family 22 member 5	0	0	0	1
Bile salt export pump	0	1	0	1
Solute carrier family 22 member 16	0	0	0	1
Solute carrier family 22 member 21	0	0	0	1
Solute carrier family 22 member 5	0	0	0	1
Chain A, Phosphomannomutase/phosphoglucomutase	0	0	1	1
Chain A, Purine-nucleoside Phosphorylase	0	1	0	1
Chain C, Xanthine dehydrogenase/oxidase	0	0	1	1
cytochrome P450 family 3 subfamily A polypeptide 4	2	0	0	2
peroxisome proliferator activated receptor gamma	2	0	0	2
activating transcription factor 6	1	0	0	1
Hypoxanthine-guanine phosphoribosyltransferase	0	1	0	2
Purine nucleoside phosphorylase	0	0	1	2
Chain A, ADENOSINE DEAMINASE	0	1	0	1
Chain A, ADENOSINE DEAMINASE	0	1	0	1
Chain A, Membrane lipoprotein tmpC	0	0	1	1
Chain A, Membrane lipoprotein tmpC	0	0	1	1
Chain A, Membrane lipoprotein tmpC	0	0	1	1
Chain A, Ferritin light chain	1	0	0	1
Smad3	1	0	0	1
bromodomain adjacent to zinc finger domain 2B	2	0	0	2
IDH1	1	0	0	1
nuclear factor erythroid 2-related factor 2 isoform 2	1	0	0	1
glycogen synthase kinase-3 beta isoform 1	0	0	1	1
geminin	1	0	0	1
survival motor neuron protein isoform d	1	0	0	1
muscleblind-like protein 1 isoform 1	1	0	0	1
Purine nucleoside phosphorylase	0	0	0	1
Adenosylhomocysteinase	0	1	0	2
Adenosine deaminase	0	1	0	1
Chain A, Hypoxanthine Phosphoribosyltransferase	0	1	0	1
Chain A, HYPOXANTHINE PHOSPHORIBOSYLTRANSFERASE	0	1	0	1
Chain A, hypoxanthine phosphoribosyltransferase	0	0	1	1
Chain A, hypoxanthine phosphoribosyltransferase	0	0	1	1
Chain A, HYPOXANTHINE-GUANINE PHOSPHORIBOSYLTRANSFERASE	0	0	1	1
Chain B, HYPOXANTHINE-GUANINE PHOSPHORIBOSYLTRANSFERASE	0	0	1	1
Chain A, HYPOXANTHINE-GUANINE PHOSPHORIBOSYLTRANSFERASE	0	0	1	1
Chain A, Hypoxanthine-guanine phosphoribosyltransferase	0	1	0	1
Inosine-5'-monophosphate dehydrogenase 2	0	1	0	2
Inosine-5'-monophosphate dehydrogenase 1	0	1	0	2
acetylcholinesterase	1	0	0	1
estrogen receptor 2 (ER beta)	1	0	0	1
retinoic acid nuclear receptor alpha variant 1	3	0	0	3
estrogen nuclear receptor alpha	2	0	0	2
cytochrome P450 2D6	1	0	0	1
thyroid stimulating hormone receptor	2	0	0	2
cellular tumor antigen p53 isoform a	1	0	0	1
Integrin beta-3	1	0	0	1
Integrin alpha-IIb	1	0	0	1
progesterone receptor	1	0	0	1
thyroid hormone receptor beta isoform 2	1	0	0	1
nuclear factor erythroid 2-related factor 2 isoform 1	1	0	0	1
Monocarboxylate transporter 4	0	0	0	1
Monocarboxylate transporter 2	0	0	0	1
Cellular tumor antigen p53	1	0	0	1
Monocarboxylate transporter 1	0	0	0	1
C-terminal-binding protein 2	0	1	0	1
Monocarboxylate transporter 2	0	1	0	1
Solute carrier family 22 member 20	0	1	0	1
Prolyl 4-hydroxylase	0	1	0	1
Solute carrier family 22 member 6	0	1	0	1
Solute carrier organic anion transporter family member 2A1	0	1	0	1
Alpha-ketoglutarate-dependent dioxygenase FTO	0	1	0	1
Chain A, Trp Rna-binding Attenuation Protein	0	0	1	1
Chain K, Trp Rna-binding Attenuation Protein	0	0	1	1
Chain B, tryptophanyl-tRNA synthetase	0	0	1	1
Chain C, Tryptophanyl-tRNA synthetase II	0	0	1	1
thioredoxin reductase	1	0	0	1
GLS protein	1	0	0	1
GLI family zinc finger 3	1	0	0	1
thioredoxin glutathione reductase	1	0	0	1
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)	1	0	0	1
thyroid stimulating hormone receptor	1	0	0	1
arylsulfatase A	1	0	0	1
aryl hydrocarbon receptor	1	0	0	1
Bloom syndrome protein isoform 1	1	0	0	1
peripheral myelin protein 22 isoform 1	1	0	0	1
flap endonuclease 1	1	0	0	1
histone-lysine N-methyltransferase 2A isoform 2 precursor	1	0	0	1
DNA polymerase iota isoform a (long)	1	0	0	1
M-phase phosphoprotein 8	1	0	0	1
Carbonic anhydrase 12	0	0	0	1
Dihydrofolate reductase	0	2	0	2
Plasma kallikrein	0	0	0	1
Myeloperoxidase	0	1	0	1
Carbonic anhydrase 3	0	0	0	1
Stromelysin-1	0	1	0	1
Intestinal-type alkaline phosphatase	0	1	0	1
Indoleamine 2,3-dioxygenase 1	0	1	0	2
Carbonic anhydrase 4	0	0	0	1
Carbonic anhydrase 6	0	0	0	1
Substance-P receptor	0	1	0	1
Carbonic anhydrase 5A, mitochondrial	0	0	0	1
Carbonic anhydrase	0	0	0	1
Carbonic anhydrase 7	0	0	0	1
Tryptophan 2,3-dioxygenase	0	1	0	2
Carbonic anhydrase	0	0	0	1
Glutamate receptor ionotropic, NMDA 2A	0	0	0	1
Glutamate receptor ionotropic, NMDA 2B	0	0	0	1
Glutamate receptor ionotropic, NMDA 2C	0	0	0	1
Large neutral amino acids transporter small subunit 1	0	1	0	1
SLC16A10 protein	0	0	0	1
Uracil nucleotide/cysteinyl leukotriene receptor	0	1	0	1
Carbonic anhydrase 9	0	0	0	1
Carbonic anhydrase	0	0	0	1
Tryprostatin B synthase	0	0	0	1
Carbonic anhydrase	0	0	0	1
Carbonic anhydrase	0	0	0	1
Glutamate receptor ionotropic, NMDA 2D	0	0	0	1
2-C-methyl-D-erythritol 2,4-cyclodiphosphate synthase	0	0	1	1
Carbonic anhydrase	0	0	0	1
Glutamate receptor ionotropic, NMDA 3B	0	0	0	1
Monocarboxylate transporter 10	0	0	0	1
Carbonic anhydrase 15	0	0	0	1
Carbonic anhydrase 13	0	0	0	1
Glutamate receptor ionotropic, NMDA 3A	0	0	0	1
Carbonic anhydrase 14	0	0	0	1
Phospholipase A-2-activating protein	0	1	0	1
Carbonic anhydrase 5B, mitochondrial	0	0	0	1
Chain A, Glycogen phosphorylase, liver form	0	0	1	1
Chain A, glycogen phosphorylase, liver form	0	0	1	1
Chain A, Glycogen phosphorylase, liver form	0	0	1	1
Chain A, Glycogen phosphorylase, liver form	0	0	1	1
Solute carrier family 22 member 6	0	0	0	1
Solute carrier family 22 member 8	0	1	0	1
Solute carrier family 2, facilitated glucose transporter member 9	0	1	1	2
Guanine deaminase	0	1	0	1
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Glycogen Storage Disease Type VII

Synonyms(1)

Research

Studies (36)

Protein Targets (266)

Glycogen Storage Disease Type VII

Synonyms(1)

Research

Studies (36)

Drugs Studied (28)

Protein Targets (266)