Ikbkg inhibitor of kappaB kinase gamma [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Ikbkgprovided by MGI
Official Full Name: inhibitor of kappaB kinase gammaprovided by MGI
Primary source: MGI:MGI:1338074
See related: Ensembl:ENSMUSG00000004221 AllianceGenome:MGI:1338074
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: NEMO; IKK[g]; 1110037D23Rik
Summary: Enables K63-linked polyubiquitin modification-dependent protein binding activity; identical protein binding activity; and signaling adaptor activity. Involved in several processes, including anoikis; canonical NF-kappaB signal transduction; and negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway. Acts upstream of or within B cell homeostasis and positive regulation of canonical NF-kappaB signal transduction. Located in cytoplasm. Part of IkappaB kinase complex. Is expressed in early conceptus; genitourinary system; and musculature. Used to study Bloch-Sulzberger syndrome and steatotic liver disease. Human ortholog(s) of this gene implicated in Behcet's disease; Bloch-Sulzberger syndrome; X-linked recessive disease (multiple); learning disability; and liver cancer. Orthologous to human IKBKG (inhibitor of nuclear factor kappa B kinase regulatory subunit gamma). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Ubiquitous expression in liver E18 (RPKM 3.8), bladder adult (RPKM 3.7) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: X A7.3; X 38.0 cM

Exon count:: 15

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	X	NC_000086.8 (73436883..73498013)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	X	NC_000086.7 (74393233..74456792)

Chromosome X - NC_000086.8 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

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

NEMO/NF-kappaB signaling functions as a double-edged sword in PanIN formation versus progression to pancreatic cancer.
Title: NEMO/NF-κB signaling functions as a double-edged sword in PanIN formation versus progression to pancreatic cancer.
USP10 regulates macrophage inflammation responses via stabilizing NEMO in LPS-induced sepsis.
Title: USP10 regulates macrophage inflammation responses via stabilizing NEMO in LPS-induced sepsis.
Damaged mitochondria recruit the effector NEMO to activate NF-kappaB signaling.
Title: Damaged mitochondria recruit the effector NEMO to activate NF-κB signaling.
NEMO- and RelA-dependent NF-kappaB signaling promotes small cell lung cancer.
Title: NEMO- and RelA-dependent NF-κB signaling promotes small cell lung cancer.
TREM-1 induces pyroptosis in cardiomyocytes by activating NLRP3 inflammasome through the SMC4/NEMO pathway.
Title: TREM-1 induces pyroptosis in cardiomyocytes by activating NLRP3 inflammasome through the SMC4/NEMO pathway.
Smooth muscle cell specific NEMO deficiency inhibits atherosclerosis in ApoE(-/-) mice.
Title: Smooth muscle cell specific NEMO deficiency inhibits atherosclerosis in ApoE(-/-) mice.
SENP1 modulates microglia-mediated neuroinflammation toward intermittent hypoxia-induced cognitive decline through the de-SUMOylation of NEMO.
Title: SENP1 modulates microglia-mediated neuroinflammation toward intermittent hypoxia-induced cognitive decline through the de-SUMOylation of NEMO.
Nemo-Dependent, ATM-Mediated Signals from RAG DNA Breaks at Igk Feedback Inhibit V kappa Recombination to Enforce Igkappa Allelic Exclusion.
Title: Nemo-Dependent, ATM-Mediated Signals from RAG DNA Breaks at Igk Feedback Inhibit V (κ) Recombination to Enforce Igκ Allelic Exclusion.
Genomic locus proteomic screening identifies the NF-kappaB signaling pathway components NFkappaB1 and IKBKG as transcriptional regulators of Ripk3 in endothelial cells.
Title: Genomic locus proteomic screening identifies the NF-κB signaling pathway components NFκB1 and IKBKG as transcriptional regulators of Ripk3 in endothelial cells.
Epsins 1 and 2 promote NEMO linear ubiquitination via LUBAC to drive breast cancer development.
Title: Epsins 1 and 2 promote NEMO linear ubiquitination via LUBAC to drive breast cancer development.

Submit: New GeneRIF Correction See all GeneRIFs (94)

Variation

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Alleles

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

Chemically induced (ENU) (1)
Endonuclease-mediated (3)
Targeted (9) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

AF069542 (e-PCR)
- UniSTS:163621

AI851264 (e-PCR)
- UniSTS:166184

AI848108 (e-PCR)
- UniSTS:166185

AI462521 (e-PCR)
- UniSTS:181300

AW124339 (e-PCR)
- UniSTS:181302

D9Mit34.1 (e-PCR), detects polymorphism
- UniSTS:131903

PMC85812P2 (e-PCR)
- UniSTS:273495

UniSTS:256936 (e-PCR)
- UniSTS:256936

G6pdx (e-PCR), detects polymorphism
- UniSTS:256936

Z11911 (e-PCR)
- UniSTS:125912

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables K63-linked polyubiquitin modification-dependent protein binding	IBA Inferred from Biological aspect of Ancestor more info
enables K63-linked polyubiquitin modification-dependent protein binding	IDA Inferred from Direct Assay more info	PubMed
enables K63-linked polyubiquitin modification-dependent protein binding	ISO Inferred from Sequence Orthology more info
enables identical protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables identical protein binding	ISO Inferred from Sequence Orthology more info
enables linear polyubiquitin binding	ISO Inferred from Sequence Orthology more info
enables linear polyubiquitin binding	ISS Inferred from Sequence or Structural Similarity more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables peroxisome proliferator activated receptor binding	ISO Inferred from Sequence Orthology more info
enables polyubiquitin modification-dependent protein binding	ISO Inferred from Sequence Orthology more info
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables protein domain specific binding	ISO Inferred from Sequence Orthology more info
enables protein heterodimerization activity	ISO Inferred from Sequence Orthology more info
enables protein heterodimerization activity	ISS Inferred from Sequence or Structural Similarity more info
enables protein homodimerization activity	ISO Inferred from Sequence Orthology more info
enables protein homodimerization activity	ISS Inferred from Sequence or Structural Similarity more info
enables protein-containing complex binding	ISO Inferred from Sequence Orthology more info
enables signaling adaptor activity	IDA Inferred from Direct Assay more info	PubMed
enables signaling adaptor activity	ISO Inferred from Sequence Orthology more info
enables transferrin receptor binding	ISO Inferred from Sequence Orthology more info
enables ubiquitin protein ligase binding	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
acts_upstream_of_or_within B cell homeostasis	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in DNA damage response	ISO Inferred from Sequence Orthology more info
involved_in DNA damage response	ISS Inferred from Sequence or Structural Similarity more info
involved_in anoikis	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in canonical NF-kappaB signal transduction	IDA Inferred from Direct Assay more info	PubMed
involved_in canonical NF-kappaB signal transduction	ISO Inferred from Sequence Orthology more info
involved_in defense response to bacterium	ISO Inferred from Sequence Orthology more info
involved_in establishment of vesicle localization	ISO Inferred from Sequence Orthology more info
involved_in negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of NF-kappaB transcription factor activity	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of NF-kappaB transcription factor activity	ISS Inferred from Sequence or Structural Similarity more info
involved_in positive regulation of T cell receptor signaling pathway	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of T cell receptor signaling pathway	ISS Inferred from Sequence or Structural Similarity more info
involved_in positive regulation of canonical NF-kappaB signal transduction	IBA Inferred from Biological aspect of Ancestor more info
acts_upstream_of_or_within positive regulation of canonical NF-kappaB signal transduction	IDA Inferred from Direct Assay more info	PubMed
acts_upstream_of_or_within positive regulation of canonical NF-kappaB signal transduction	ISO Inferred from Sequence Orthology more info	PubMed
involved_in positive regulation of canonical NF-kappaB signal transduction	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of canonical NF-kappaB signal transduction	ISS Inferred from Sequence or Structural Similarity more info
involved_in positive regulation of gene expression	IGI Inferred from Genetic Interaction more info	PubMed
involved_in positive regulation of macroautophagy	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of transcription by RNA polymerase II	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of transcription by RNA polymerase II	ISS Inferred from Sequence or Structural Similarity more info
involved_in protein-containing complex assembly	ISO Inferred from Sequence Orthology more info
involved_in tumor necrosis factor-mediated signaling pathway	TAS Traceable Author Statement more info	PubMed

Component	Evidence Code	Pubs
part_of IkappaB kinase complex	IBA Inferred from Biological aspect of Ancestor more info
part_of IkappaB kinase complex	IPI Inferred from Physical Interaction more info	PubMed
part_of IkappaB kinase complex	ISO Inferred from Sequence Orthology more info	PubMed
is_active_in cytoplasm	IBA Inferred from Biological aspect of Ancestor more info
located_in cytoplasm	IDA Inferred from Direct Assay more info	PubMed
located_in cytoplasm	ISO Inferred from Sequence Orthology more info
is_active_in cytosol	ISO Inferred from Sequence Orthology more info
located_in cytosol	ISO Inferred from Sequence Orthology more info
located_in mitotic spindle	ISO Inferred from Sequence Orthology more info
is_active_in nucleus	IBA Inferred from Biological aspect of Ancestor more info
located_in nucleus	IEA Inferred from Electronic Annotation more info
located_in nucleus	ISO Inferred from Sequence Orthology more info
part_of protein-containing complex	ISO Inferred from Sequence Orthology more info
located_in spindle pole	ISO Inferred from Sequence Orthology more info
part_of ubiquitin ligase complex	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: NF-kappa-B essential modulator

Names: I-kappa-B kinase subunit gamma; IKK-gamma; IKKAP1; IKKG; NF-kappa-B essential modifier; ikB kinase subunit gamma; ikB kinase-associated protein 1; inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma; inhibitor of nuclear factor kappa-B kinase subunit gamma; mFIP-3

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_001136067.2 → NP_001129539.1 NF-kappa-B essential modulator isoform 3

See identical proteins and their annotated locations for NP_001129539.1

Status: VALIDATED

Description

Transcript Variant: This variant (3) represents the longest transcript and encodes the longest isoform (3).

Source sequence(s)

AL669976, AY112937

Consensus CDS

CCDS53108.1

UniProtKB/TrEMBL

Q7TSS3

Related

ENSMUSP00000004330.4, ENSMUST00000004330.10

Conserved Domains (4) summary

cd09803
Location:269 → 355

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:67 → 303

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:62 → 128

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:404 → 429

zf_C2H2_10; C2H2 type zinc-finger
NM_001161421.1 → NP_001154893.1 NF-kappa-B essential modulator isoform 4

Status: VALIDATED

Description

Transcript Variant: This variant (4) uses an alternate in-frame splice junction at the 5' end of an exon compared to variant 3. The resulting isoform (4) has the same N- and C-termini but is 1 aa shorter compared to isoform 3.

Source sequence(s)

AK037020, AL669976, AY112937

UniProtKB/TrEMBL

Q7TSS3, Q8VC91

Conserved Domains (3) summary

cd09803
Location:268 → 354

UBAN; polyubiquitin binding domain of NEMO and related proteins

pfam11577
Location:62 → 129

NEMO; NF-kappa-B essential modulator NEMO

cl00459
Location:185 → 297

MIT_CorA-like; metal ion transporter CorA-like divalent cation transporter superfamily
NM_001161422.1 → NP_001154894.1 NF-kappa-B essential modulator isoform 5

Status: VALIDATED

Description

Transcript Variant: This variant (5) differs in the 5' UTR and coding sequence compared to variant 3. The resulting isoform (5) has a shorter and distinct N-terminus compared to isoform 3.

Source sequence(s)

AL669976, AY112937, CN701499

Consensus CDS

CCDS53109.1

UniProtKB/TrEMBL

E9Q2Y3

Related

ENSMUSP00000109765.3, ENSMUST00000114130.9

Conserved Domains (3) summary

cd09803
Location:263 → 349

UBAN; polyubiquitin binding domain of NEMO and related proteins

pfam11577
Location:56 → 123

NEMO; NF-kappa-B essential modulator NEMO

cl00459
Location:180 → 292

MIT_CorA-like; metal ion transporter CorA-like divalent cation transporter superfamily
NM_001161423.1 → NP_001154895.1 NF-kappa-B essential modulator isoform 1

See identical proteins and their annotated locations for NP_001154895.1

Status: VALIDATED

Description

Transcript Variant: This variant (6) differs in the 5' UTR and coding sequence compared to variant 3. The resulting isoform (1) is shorter at the N-terminus compared to isoform 3. Variants 1 and 6 both encode the same isoform.

Source sequence(s)

AK037020, AL669976, CJ099503, CN717929

Consensus CDS

CCDS41023.1

UniProtKB/Swiss-Prot

O88522, Q924H4

Related

ENSMUSP00000126770.2, ENSMUST00000164101.8

Conserved Domains (4) summary

cd09803
Location:251 → 337

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:49 → 285

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:44 → 110

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:386 → 411

zf_C2H2_10; C2H2 type zinc-finger
NM_001161424.1 → NP_001154896.1 NF-kappa-B essential modulator isoform 2

See identical proteins and their annotated locations for NP_001154896.1

Status: VALIDATED

Description

Transcript Variant: This variant (7) differs in the 5' UTR and coding sequence and uses an alternate in-frame splice junction at the 5' end of an exon compared to variant 3. The resulting isoform (2) is shorter at the N-terminus and lacks an internal aa compared to isoform 3. Variants 2 and 7 both encode the same isoform.

Source sequence(s)

AK037020, AL669976, CJ099503, CV556906

Consensus CDS

CCDS30233.1

UniProtKB/TrEMBL

Q8VC91

Related

ENSMUSP00000136573.2, ENSMUST00000180230.8

Conserved Domains (4) summary

cd09803
Location:250 → 336

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:49 → 284

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:44 → 110

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:385 → 410

zf_C2H2_10; C2H2 type zinc-finger
NM_010547.2 → NP_034677.2 NF-kappa-B essential modulator isoform 1

See identical proteins and their annotated locations for NP_034677.2

Status: VALIDATED

Description

Transcript Variant: This variant (1) differs in the 5' UTR and coding sequence compared to variant 3. The resulting isoform (1) is shorter at the N-terminus compared to isoform 3. Variants 1 and 6 both encode the same isoform.

Source sequence(s)

AL669976, AY112937, BY768708

Consensus CDS

CCDS41023.1

UniProtKB/Swiss-Prot

O88522, Q924H4

Related

ENSMUSP00000109762.2, ENSMUST00000114127.8

Conserved Domains (4) summary

cd09803
Location:251 → 337

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:49 → 285

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:44 → 110

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:386 → 411

zf_C2H2_10; C2H2 type zinc-finger
NM_178590.4 → NP_848705.1 NF-kappa-B essential modulator isoform 2

See identical proteins and their annotated locations for NP_848705.1

Status: VALIDATED

Description

Transcript Variant: This variant (2) differs in the 5' UTR and coding sequence and uses an alternate in-frame splice junction at the 5' end of an exon compared to variant 3. The resulting isoform (2) is shorter at the N-terminus and lacks an internal aa compared to isoform 3. Variants 2 and 7 both encode the same isoform.

Source sequence(s)

AK037020, AL669976, BY768708

Consensus CDS

CCDS30233.1

UniProtKB/TrEMBL

Q8VC91

Related

ENSMUSP00000064017.4, ENSMUST00000064407.10

Conserved Domains (4) summary

cd09803
Location:250 → 336

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:49 → 284

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:44 → 110

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:385 → 410

zf_C2H2_10; C2H2 type zinc-finger

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_000086.8 Reference GRCm39 C57BL/6J

Range

73436883..73498013

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_006527848.5 → XP_006527911.1 NF-kappa-B essential modulator isoform X1

See identical proteins and their annotated locations for XP_006527911.1

UniProtKB/TrEMBL

Q7TSS3

Conserved Domains (4) summary

cd09803
Location:269 → 355

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:67 → 303

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:62 → 128

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:404 → 429

zf_C2H2_10; C2H2 type zinc-finger
XM_006527852.4 → XP_006527915.1 NF-kappa-B essential modulator isoform X3

See identical proteins and their annotated locations for XP_006527915.1

UniProtKB/Swiss-Prot

O88522, Q924H4

Conserved Domains (4) summary

cd09803
Location:251 → 337

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:49 → 285

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:44 → 110

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:386 → 411

zf_C2H2_10; C2H2 type zinc-finger
XM_006527853.5 → XP_006527916.1 NF-kappa-B essential modulator isoform X4

See identical proteins and their annotated locations for XP_006527916.1

UniProtKB/TrEMBL

Q8VC91

Related

ENSMUSP00000109764.3, ENSMUST00000114129.9

Conserved Domains (4) summary

cd09803
Location:250 → 336

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:49 → 284

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:44 → 110

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:385 → 410

zf_C2H2_10; C2H2 type zinc-finger
XM_006527847.5 → XP_006527910.1 NF-kappa-B essential modulator isoform X1

See identical proteins and their annotated locations for XP_006527910.1

UniProtKB/TrEMBL

Q7TSS3

Conserved Domains (4) summary

cd09803
Location:269 → 355

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:67 → 303

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:62 → 128

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:404 → 429

zf_C2H2_10; C2H2 type zinc-finger
XM_006527850.4 → XP_006527913.1 NF-kappa-B essential modulator isoform X3

See identical proteins and their annotated locations for XP_006527913.1

UniProtKB/Swiss-Prot

O88522, Q924H4

Related

ENSMUSP00000109763.2, ENSMUST00000114128.8

Conserved Domains (4) summary

cd09803
Location:251 → 337

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:49 → 285

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:44 → 110

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:386 → 411

zf_C2H2_10; C2H2 type zinc-finger
XM_006527851.4 → XP_006527914.1 NF-kappa-B essential modulator isoform X3

See identical proteins and their annotated locations for XP_006527914.1

UniProtKB/Swiss-Prot

O88522, Q924H4

Conserved Domains (4) summary

cd09803
Location:251 → 337

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:49 → 285

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:44 → 110

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:386 → 411

zf_C2H2_10; C2H2 type zinc-finger
XM_006527849.3 → XP_006527912.1 NF-kappa-B essential modulator isoform X2

Conserved Domains (4) summary

cd09803
Location:257 → 343

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:55 → 291

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:50 → 116

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:392 → 417

zf_C2H2_10; C2H2 type zinc-finger
XM_011247527.4 → XP_011245829.1 NF-kappa-B essential modulator isoform X3

See identical proteins and their annotated locations for XP_011245829.1

UniProtKB/Swiss-Prot

O88522, Q924H4

Conserved Domains (4) summary

cd09803
Location:251 → 337

UBAN; polyubiquitin binding domain of NEMO and related proteins

TIGR02168
Location:49 → 285

SMC_prok_B; chromosome segregation protein SMC, common bacterial type

pfam11577
Location:44 → 110

NEMO; NF-kappa-B essential modulator NEMO

pfam18414
Location:386 → 411

zf_C2H2_10; C2H2 type zinc-finger