DERL3 (derlin 3)

Gene

• Entrez ID: 91319
• Gene name: Derlin 3
• Gene symbol: DERL3
• Synonyms: C22orf14, IZP6, LLN2, derlin-3
• Chromosome: 22
• Chromosome location: 22q11.23
• Summary: The protein encoded by this gene belongs to the derlin family, and resides in the endoplasmic reticulum (ER). Proteins that are unfolded or misfolded in the ER must be refolded or degraded to maintain the homeostasis of the ER. This protein appears to be involved in the degradation of misfolded glycoproteins in the ER. Several alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Oct 2008]

Gene ontology (GO)

GO ID	Ontology	Definition	Evidence	Reference
GO:0000839	Component	Hrd1p ubiquitin ligase ERAD-L complex	IBA	21873635
GO:0005047	Function	Signal recognition particle binding	IDA	26565908
GO:0005515	Function	Protein binding	IPI	16449189, 19135427, 32296183
GO:0018279	Process	Protein N-linked glycosylation via asparagine	IMP	22607976
GO:0030176	Component	Integral component of endoplasmic reticulum membrane	IBA	21873635
GO:0030176	Component	Integral component of endoplasmic reticulum membrane	IDA	16449189
GO:0030433	Process	Ubiquitin-dependent ERAD pathway	IBA	21873635
GO:0030433	Process	Ubiquitin-dependent ERAD pathway	IMP	22607976
GO:0030433	Process	Ubiquitin-dependent ERAD pathway	NAS	16449189
GO:0030433	Process	Ubiquitin-dependent ERAD pathway	TAS	22013210
GO:0030968	Process	Endoplasmic reticulum unfolded protein response	IBA	21873635
GO:0030968	Process	Endoplasmic reticulum unfolded protein response	IDA	16449189
GO:0044877	Function	Protein-containing complex binding	IPI	26565908
GO:0051787	Function	Misfolded protein binding	IBA	21873635
GO:1904153	Process	Negative regulation of retrograde protein transport, ER to cytosol	IMP	25660456
GO:1990381	Function	Ubiquitin-specific protease binding	IBA	21873635

Other IDs

• MIM: 610305
• HGNC: 14236
• e!Ensembl: ENSG00000099958

Protein

• UniProt ID: Q96Q80
• Protein name: Derlin-3 (Degradation in endoplasmic reticulum protein 3) (DERtrin-3) (Der1-like protein 3)
• Protein function: Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal glycoproteins, but not that of misfolded nonglycoproteins. May act by forming a channel that allows the retrotranslocation of misfolded glycoproteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and the misfolded glycoproteins (PubMed:16449189, PubMed:22607976). May be involved in endoplasmic reticulum stress-induced pre-emptive quality control, a mechanism that selectively attenuates the translocation of newly synthesized proteins into the endoplasmic reticulum and reroutes them to the cytosol for proteasomal degradation (PubMed:26565908).
• Family and domains:

  Pfam

  Accession	ID	Position in sequence	Description	Type
  PF04511	DER1	13 → 203	Der1-like family	Family

• Sequence:

  MAWQGLAAEFLQVPAVTRAYTAACVLTTAAVQLELLSPFQLYFNPHLVFRKFQVWRLVTN
  FLFFGPLGFSFFFNMLFVFRYCRMLEEGSFRGRTADFVFMFLFGGVLMTLLGLLGSLFFL
  GQALMAMLVYVWSRRSPRVRVNFFGLLTFQAPFLPWALMGFSLLLGNSILVDLLGIAVGH
  IYYFLEDVFPNQPGGKRLLQTPGFLKLLLDAPAEDPNYLPLPEEQPGPHLPPPQQ

• Sequence length: 235