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Proteins/UNIPROT:Q6ZXV5

TMTC3

Protein·UNIPROT:Q6ZXV5

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Transfers mannosyl residues to the hydroxyl group of serine or threonine residues. The 4 members of the TMTC family are O-mannosyl-transferases dedicated primarily to the cadherin superfamily, each member seems to have a distinct role in decorating the cadherin domains with O-linked mannose glycans at specific regions. Also acts as O-mannosyl-transferase on other proteins such as PDIA3. Involved in the positive regulation of proteasomal protein degradation in the endoplasmic reticulum (ER), and the control of ER stress response..

Function from UniProtKB (Q6ZXV5), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Inferred

Inferred — not reported by a source. Evidence key

GenCC submitters' classifications

2 rows

lissencephaly 8 has a germline basis in TMTC3DiseaseGenCC HGNC:26899|MONDO:0014992Inferred only
periventricular nodular heterotopia has a germline basis in TMTC3DiseaseGenCC HGNC:26899|MONDO:0020341Inferred only

Statements

1 statement

1 statement · 1 reference database · Subject: TMTC3 · Sources: Hetionet

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UBC interacts with TMTC3ProteinHetionet hetionet:interacts:160418-7316Inferred only

Reactions

2 reactions

a di-trans,poly-cis-dolichyl beta-D-mannosyl phosphate+L-seryl-[protein]3-O-(alpha-D-mannosyl)-L-seryl-[protein]+a di-trans,poly-cis-dolichyl phosphate+hydron
Catalysed by TMTC3 or TMTC1 or TMTC2 or TMEM260 or POMT2 or POMT1
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:17377CC BY 4.0Reference data
a di-trans,poly-cis-dolichyl beta-D-mannosyl phosphate+L-threonyl-[protein]3-O-(alpha-D-mannosyl)-L-threonyl-[protein]

Other names

4 names

Resolves to: TMTC3

Also called: Protein SMILESMILETransmembrane and tetratricopeptide repeat-containing 3Transmembrane O-mannosyltransferase targeting cadherins 3

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+a di-trans,poly-cis-dolichyl phosphate

Catalysed by TMTC3 or TMTC1 or TMTC2 or TMEM260 or POMT2 or POMT1

Reaction pageLeft to rightBalance undeterminable

Rhea RHEA:53396CC BY 4.0Reference data