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PYGM | phosphorylase, glycogen, muscle; 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 (By similarity) (842 aa) | |||
PYGB | phosphorylase, glycogen; brain; 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 (By similarity) (843 aa) | |||
PGRMC1 | progesterone receptor membrane component 1; Receptor for progesterone (By similarity) (195 aa) | |||
PPP1R3C | protein phosphatase 1, regulatory subunit 3C; Acts as a glycogen-targeting subunit for PP1 and regulates its activity. Activates glycogen synthase, reduces glycogen phosphorylase activity and limits glycogen breakdown. Dramatically increases basal and insulin-stimulated glycogen synthesis upon overexpression in a variety of cell types (317 aa) | |||
POM121 | POM121 transmembrane nucleoporin (984 aa) | |||
GYS2 | glycogen synthase 2 (liver); Transfers the glycosyl residue from UDP-Glc to the non- reducing end of alpha-1,4-glucan (703 aa) | |||
ALG1 | asparagine-linked glycosylation 1, beta-1,4-mannosyltransferase homolog (S. cerevisiae); Participates in the formation of the lipid-linked precursor oligosaccharide for N-glycosylation. Involved in assembling the dolichol-pyrophosphate-GlcNAc(2)-Man(5) intermediate on the cytoplasmic surface of the ER (464 aa) | |||
TRIM7 | tripartite motif containing 7 (511 aa) | |||
AGL | amylo-alpha-1, 6-glucosidase, 4-alpha-glucanotransferase (1532 aa) | |||
PHKG1 | phosphorylase kinase, gamma 1 (muscle); Catalytic subunit of the phosphorylase b kinase (PHK), which mediates the neural and hormonal regulation of glycogen breakdown (glycogenolysis) by phosphorylating and thereby activating glycogen phosphorylase. In vitro, phosphorylates PYGM, TNNI3, MAPT/TAU, GAP43 and NRGN/RC3 (By similarity) (387 aa) | |||
PHKB | phosphorylase kinase, beta; Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The beta chain acts as a regulatory unit and modulates the activity of the holoenzyme in response to phosphorylation (1093 aa) | |||
GYS1 | glycogen synthase 1 (muscle); Transfers the glycosyl residue from UDP-Glc to the non- reducing end of alpha-1,4-glucan (737 aa) | |||
UGP2 | UDP-glucose pyrophosphorylase 2; Plays a central role as a glucosyl donor in cellular metabolic pathways (508 aa) | |||
ALG1L | asparagine-linked glycosylation 1-like; Putative glycosyltransferase (By similarity) (187 aa) | |||
GYG1 | glycogenin 1; Self-glucosylates, via an inter-subunit mechanism, to form an oligosaccharide primer that serves as substrate for glycogen synthase (350 aa) | |||
UBC | ubiquitin C (685 aa) | |||
NHLRC1 | NHL repeat containing 1; E3 ubiquitin-protein ligase which in complex with EPM2A/laforin and HSP70 suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Ubiquitinates PPP1R3C/PTG in a laforin-dependent manner, and targets it for proteasome-dependent degradation and this degradation decreases glycogen accumulation. Polyubiquitinates EPM2A/laforin and ubiquitinates AGL and targets them for proteasome-dependent degradation (395 aa) | |||
NENF | neudesin neurotrophic factor; Displays neurotrophic activity and activates phosphorylation of MAPK1/ERK2, MAPK3/ERK1 and AKT1/AKT in primary cultured neurons. Does not have mitogenic activity in primary cultured astrocytes. May play a role on neuronal differentiation and may have a transient effect on neural cell proliferation in neural precursor cells. Neurotrophic activity is enhanced by binding to heme (By similarity) (172 aa) | |||
EPM2A | epilepsy, progressive myoclonus type 2A, Lafora disease (laforin) (331 aa) | |||
ATG2A | autophagy related 2A; Required for both autophagosome formation and regulation of lipid droplet morphology and dispersion (1938 aa) | |||
PIGA | phosphatidylinositol glycan anchor biosynthesis, class A; Necessary for the synthesis of N-acetylglucosaminyl- phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis (484 aa) | |||
GYG2 | glycogenin 2; Self-glucosylates, via an inter-subunit mechanism, to form an oligosaccharide primer that serves as substrate for glycogen synthase (501 aa) | |||
GBE1 | glucan (1,4-alpha-), branching enzyme 1; Required for sufficient glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule and, consequently, in reducing the osmotic pressure within cells (702 aa) | |||
ALG2 | asparagine-linked glycosylation 2, alpha-1,3-mannosyltransferase homolog (S. cerevisiae); Mannosylates Man(2)GlcNAc(2)-dolichol diphosphate and Man(1)GlcNAc(2)-dolichol diphosphate to form Man(3)GlcNAc(2)- dolichol diphosphate (416 aa) | |||
PGRMC2 | progesterone receptor membrane component 2; Receptor for steroids (Potential) (247 aa) | |||
ALG11 | asparagine-linked glycosylation 11, alpha-1,2-mannosyltransferase homolog (yeast); Mannosyltransferase involved in the last steps of the synthesis of Man5GlcNAc(2)-PP-dolichol core oligosaccharide on the cytoplasmic face of the endoplasmic reticulum. Catalyzes the addition of the 4th and 5th mannose residues to the dolichol- linked oligosaccharide chain (492 aa) |