Prkag2 protein kinase, AMP-activated, gamma 2 non-catalytic subunit [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Prkag2provided by MGI
Official Full Name: protein kinase, AMP-activated, gamma 2 non-catalytic subunitprovided by MGI
Primary source: MGI:MGI:1336153
See related: Ensembl:ENSMUSG00000028944 AllianceGenome:MGI:1336153
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: AAKG; WPWS; AAKG2; H91620p; 2410051C13Rik
Summary: Predicted to enable adenyl ribonucleotide binding activity; protein kinase binding activity; and protein kinase regulator activity. Predicted to contribute to AMP-activated protein kinase activity. Predicted to be involved in several processes, including cellular response to glucose starvation; glycogen metabolic process; and regulation of phosphorylation. Predicted to act upstream of or within fatty acid biosynthetic process. Predicted to be located in cytosol and nucleoplasm. Predicted to be part of nucleotide-activated protein kinase complex. Predicted to be active in cytoplasm and nucleus. Is expressed in several structures, including alimentary system; heart and pericardium; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in Wolff-Parkinson-White syndrome; glycogen storage disease; hypertrophic cardiomyopathy 6; and lethal congenital glycogen storage disease of heart. Orthologous to human PRKAG2 (protein kinase AMP-activated non-catalytic subunit gamma 2). [provided by Alliance of Genome Resources, Apr 2022]
Expression: Ubiquitous expression in bladder adult (RPKM 21.5), testis adult (RPKM 20.9) and 26 other tissues See more
Orthologs: human all
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Genomic context

Location:: 5 A3; 5 11.93 cM

Exon count:: 22

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	5	NC_000071.7 (25067733..25311576, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	5	NC_000071.6 (24862735..25106282, complement)

Chromosome 5 - NC_000071.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

gamma2 AMPK activation downregulates fundamental sinoatrial cell pacemaker mechanisms to lower heart rate, including sarcolemmal hyperpolarization-activated current (I f) and ryanodine receptor-derived diastolic local subsarcolemmal Ca(2+) release. In contrast, loss of gamma2 AMPK induces a reciprocal phenotype of increased heart rate, and prevents the adaptive intrinsic bradycardia of endurance training.
Title: Mammalian γ2 AMPK regulates intrinsic heart rate.
gamma2-specific AMPK activity was elevated in neonatal FNIP1-deficient myocardium, supporting a role for FNIP1 as a negative regulator of AMPK.
Title: Mutation of Fnip1 is associated with B-cell deficiency, cardiomyopathy, and elevated AMPK activity.
Increased AMPK-gamma2 activation is required to protect against myocardial ischemia/reperfusion injury.
Title: Activation of γ2-AMPK Suppresses Ribosome Biogenesis and Protects Against Myocardial Ischemia/Reperfusion Injury.
mice with chronic AMPK activation, resulting from mutation of the AMPK gamma2 subunit, exhibit ghrelin signaling-dependent hyperphagia, obesity, and impaired pancreatic islet insulin secretion. Humans bearing the homologous mutation manifest a congruent phenotype.
Title: Chronic Activation of γ2 AMPK Induces Obesity and Reduces β Cell Function.
These results demonstrate that expression of AMPK subunit gamma-2(NI) and AMPK subunit gamma-2(RG) mutations at physiological levels can induce beneficial metabolic effects but that this is accompanied by Wolff-Parkinson-White Syndrome syndrome.
Title: Physiological Expression of AMPKγ2RG Mutation Causes Wolff-Parkinson-White Syndrome and Induces Kidney Injury in Mice.
Prkag2 point mutation causes glycogen storage via enhanced insulin sensitivity and AKT activation. It stimulates postnatal cardiomyocyte proliferation by downregulating FoxO. It promotes cardiac hypertrophy via mTOR activation in developed hearts.
Title: Mutation in the γ2-subunit of AMP-activated protein kinase stimulates cardiomyocyte proliferation and hypertrophy independent of glycogen storage.
We have demonstrated that a third AMPK gamma2 variant, gamma2-3B, is increasingly expressed in the developing heart.
Title: Embryonic expression of AMPK γ subunits and the identification of a novel γ2 transcript variant in adult heart.
important for cigarette smoking-induced IL-8 production by lung epithelial cells
Title: Novel role of AMP-activated protein kinase signaling in cigarette smoke induction of IL-8 in human lung epithelial cells and lung inflammation in mice.
Ceramide- and AMPK-mediated signaling pathways augment the expression and activity of 11beta-HSD1 in preadipocytes by way of C/EBPbeta.
Title: Ceramide and adenosine 5'-monophosphate-activated protein kinase are two novel regulators of 11beta-hydroxysteroid dehydrogenase type 1 expression and activity in cultured preadipocytes.
Despite high glycogen content, the TGT400N heart is not protected against ischemia-reperfusion injury.
Title: A PRKAG2 mutation causes biphasic changes in myocardial AMPK activity and does not protect against ischemia.

Submit: New GeneRIF Correction See all GeneRIFs (12)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Targeted (5) 1 citation
Endonuclease-mediated (5) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

36.MMHAP17FRD12.seq (e-PCR)
- UniSTS:124173

D5Mit74 (e-PCR), detects polymorphism
- UniSTS:130613

STS-H64261 (e-PCR)
- UniSTS:23119

NoName (e-PCR)
- UniSTS:548495

NoName (e-PCR)
- UniSTS:548496

NoName (e-PCR)
- UniSTS:548497

NoName (e-PCR)
- UniSTS:548498

NoName (e-PCR)
- UniSTS:548499

NoName (e-PCR)
- UniSTS:548500

NoName (e-PCR)
- UniSTS:548501

NoName (e-PCR)
- UniSTS:548502

NoName (e-PCR)
- UniSTS:548503

NoName (e-PCR)
- UniSTS:548504

AI854673 (e-PCR)
- UniSTS:179133

D5Mit348 (e-PCR), detects polymorphism
- UniSTS:130499

D5Mit349 (e-PCR), detects polymorphism
- UniSTS:130500

23.MMHAP10FH2.seq (e-PCR)
- UniSTS:123589

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables ADP binding	ISO Inferred from Sequence Orthology more info
enables AMP binding	IBA Inferred from Biological aspect of Ancestor more info
enables AMP binding	ISO Inferred from Sequence Orthology more info
contributes_to AMP-activated protein kinase activity	ISO Inferred from Sequence Orthology more info
enables AMP-activated protein kinase activity	TAS Traceable Author Statement more info	PubMed
enables ATP binding	ISO Inferred from Sequence Orthology more info
enables cAMP-dependent protein kinase inhibitor activity	ISO Inferred from Sequence Orthology more info
enables cAMP-dependent protein kinase regulator activity	ISO Inferred from Sequence Orthology more info
enables nucleotide binding	IEA Inferred from Electronic Annotation more info
enables phosphorylase kinase regulator activity	ISO Inferred from Sequence Orthology more info
enables protein kinase activator activity	ISO Inferred from Sequence Orthology more info
enables protein kinase binding	IBA Inferred from Biological aspect of Ancestor more info
enables protein kinase binding	ISO Inferred from Sequence Orthology more info
enables protein kinase regulator activity	IBA Inferred from Biological aspect of Ancestor more info
enables protein kinase regulator activity	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
acts_upstream_of_or_within fatty acid biosynthetic process	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within fatty acid metabolic process	IEA Inferred from Electronic Annotation more info
involved_in glycogen metabolic process	ISO Inferred from Sequence Orthology more info
involved_in intracellular signal transduction	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within lipid metabolic process	IEA Inferred from Electronic Annotation more info
involved_in negative regulation of protein kinase activity	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of peptidyl-threonine phosphorylation	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of protein kinase activity	ISO Inferred from Sequence Orthology more info
involved_in protein phosphorylation	IBA Inferred from Biological aspect of Ancestor more info
involved_in regulation of catalytic activity	IBA Inferred from Biological aspect of Ancestor more info
involved_in regulation of fatty acid metabolic process	ISO Inferred from Sequence Orthology more info
involved_in regulation of glycolytic process	ISO Inferred from Sequence Orthology more info

Component	Evidence Code	Pubs
is_active_in cytoplasm	IBA Inferred from Biological aspect of Ancestor more info
located_in cytosol	TAS Traceable Author Statement more info
located_in nucleoplasm	TAS Traceable Author Statement more info
part_of nucleotide-activated protein kinase complex	IBA Inferred from Biological aspect of Ancestor more info
part_of nucleotide-activated protein kinase complex	ISO Inferred from Sequence Orthology more info
is_active_in nucleus	IBA Inferred from Biological aspect of Ancestor more info

General protein information

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Preferred Names: 5'-AMP-activated protein kinase subunit gamma-2

Names: AMPK gamma-2 chain; AMPK subunit gamma-2

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_001170555.1 → NP_001164026.1 5'-AMP-activated protein kinase subunit gamma-2 isoform 2

Status: VALIDATED

Description

Transcript Variant: This variant (2) differs in the 5' UTR, lacks a large portion of the 5' coding region, and contains an alternate in-frame splice site compared to variant 1. It initiates translation at a downstream start codon. The encoded isoform (2) is shorter than isoform 1.

Source sequence(s)

AC125270, AK032238, BQ572978, BU707674, BY128345

UniProtKB/TrEMBL

Q8BM63

Conserved Domains (2) summary

cd04618
Location:27 → 164

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:190 → 313

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase
NM_001170556.1 → NP_001164027.1 5'-AMP-activated protein kinase subunit gamma-2 isoform 3

See identical proteins and their annotated locations for NP_001164027.1

Status: VALIDATED

Description

Transcript Variant: This variant (3) differs in the 5' UTR and lacks a large portion of the 5' coding region compared to variant 1. It initiates translation at a downstream start codon. The encoded isoform (3) has a shorter N-terminus than isoform 1.

Source sequence(s)

AC125270, AK032238, AY348864, BY128345

Consensus CDS

CCDS51437.1

UniProtKB/TrEMBL

Q3TMN8, Q8BM63

Related

ENSMUSP00000110626.2, ENSMUST00000114975.8

Conserved Domains (2) summary

cd04618
Location:26 → 163

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:189 → 312

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase
NM_001310480.1 → NP_001297409.1 5'-AMP-activated protein kinase subunit gamma-2 isoform 4

See identical proteins and their annotated locations for NP_001297409.1

Status: VALIDATED

Description

Transcript Variant: This variant (4) differs in the 5' UTR, lacks a portion of the 5' coding region, and contains an alternate in-frame splice site compared to variant 1. It initiates translation at an alternate start codon. The encoded isoform (4) has a distinct N-terminus and is shorter than isoform 1.

Source sequence(s)

AC116151, AC125270, AK032238

Consensus CDS

CCDS80231.1

UniProtKB/TrEMBL

Q8BIQ9

Related

ENSMUSP00000075651.6, ENSMUST00000076306.12

Conserved Domains (3) summary

COG0517
Location:309 → 425

CBS; CBS domain [Signal transduction mechanisms]

cd04618
Location:152 → 281

CBS_pair_5; The CBS domain, named after human CBS, is a small domain originally identified in cystathionine beta-synthase and is subsequently found in a wide range of different proteins. CBS domains usually occur in tandem repeats. They associate to form a so-called ...

cd04641
Location:308 → 427

CBS_pair_28; The CBS domain, named after human CBS, is a small domain originally identified in cystathionine beta-synthase and is subsequently found in a wide range of different proteins. CBS domains usually occur in tandem repeats. They associate to form a so-called ...
NM_145401.2 → NP_663376.2 5'-AMP-activated protein kinase subunit gamma-2 isoform 1

See identical proteins and their annotated locations for NP_663376.2

Status: VALIDATED

Description

Transcript Variant: This variant (1) represents the longest transcript and encodes the longest isoform (1).

Source sequence(s)

AC116151, AC125270

Consensus CDS

CCDS19130.1

UniProtKB/Swiss-Prot

E9QK80, Q6V7V5, Q91WG5

UniProtKB/TrEMBL

Q3U4D5

Related

ENSMUSP00000030784.8, ENSMUST00000030784.14

Conserved Domains (3) summary

COG0517
Location:432 → 548

CBS; CBS domain [Signal transduction mechanisms]

cd04618
Location:275 → 404

CBS_pair_5; The CBS domain, named after human CBS, is a small domain originally identified in cystathionine beta-synthase and is subsequently found in a wide range of different proteins. CBS domains usually occur in tandem repeats. They associate to form a so-called ...

cd04641
Location:431 → 550

CBS_pair_28; The CBS domain, named after human CBS, is a small domain originally identified in cystathionine beta-synthase and is subsequently found in a wide range of different proteins. CBS domains usually occur in tandem repeats. They associate to form a so-called ...

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

NC_000071.7 Reference GRCm39 C57BL/6J

Range

25067733..25311576 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_036164724.1 → XP_036020617.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X5

UniProtKB/TrEMBL

Q3U4D5

Conserved Domains (3) summary

cd02205
Location:274 → 346

CBS_pair_SF; CBS repeat [structural motif]

cd04641
Location:353 → 476

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase

cl15354
Location:266 → 391

CBS_pair_SF; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains superfamily
XM_017320595.3 → XP_017176084.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X4

UniProtKB/TrEMBL

Q3U4D5

Conserved Domains (3) summary

cd02205
Location:275 → 347

CBS_pair_SF; CBS repeat [structural motif]

cd04641
Location:354 → 477

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase

cl15354
Location:267 → 392

CBS_pair_SF; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains superfamily
XM_006535611.5 → XP_006535674.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X1

UniProtKB/TrEMBL

Q3U4D5

Conserved Domains (2) summary

cd04618
Location:267 → 404

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:430 → 553

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase
XM_006535612.5 → XP_006535675.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X2

UniProtKB/TrEMBL

Q3U4D5

Conserved Domains (2) summary

cd04618
Location:223 → 360

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:386 → 509

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase
XM_030254043.2 → XP_030109903.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X3

UniProtKB/TrEMBL

Q3U4D5

Conserved Domains (2) summary

cd04618
Location:222 → 359

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:385 → 508

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase
XM_006535614.4 → XP_006535677.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X6

Conserved Domains (2) summary

cd04618
Location:115 → 252

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:278 → 401

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase
XM_030254044.2 → XP_030109904.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X9

UniProtKB/TrEMBL

Q8BM63

Conserved Domains (2) summary

cd04618
Location:8 → 145

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:171 → 294

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase
XM_011249740.2 → XP_011248042.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X8

UniProtKB/TrEMBL

Q8BM63

Conserved Domains (2) summary

cd04618
Location:24 → 161

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:187 → 310

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase
XM_017320596.2 → XP_017176085.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X7

UniProtKB/TrEMBL

Q8BM63

Conserved Domains (2) summary

cd04618
Location:25 → 162

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:188 → 311

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase
XM_030254045.1 → XP_030109905.1 5'-AMP-activated protein kinase subunit gamma-2 isoform X9

UniProtKB/TrEMBL

Q8BM63

Conserved Domains (2) summary

cd04618
Location:8 → 145

CBS_euAMPK_gamma-like_repeat1; Two tandem repeats of the cystathionine beta-synthase (CBS pair) domains found in AMP-activated protein kinase gamma-like proteins, repeat 1

cd04641
Location:171 → 294

CBS_euAMPK_gamma-like_repeat2; CBS pair domain found in 5'-AMP (adenosine monophosphate)-activated protein kinase