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Structure paper

TitleCa-mediated higher-order assembly of heterodimers in amino acid transport system b biogenesis and cystinuria.
Journal, issue, pagesNat Commun, Vol. 13, Issue 1, Page 2708, Year 2022
Publish dateMay 16, 2022
AuthorsYongchan Lee / Pattama Wiriyasermkul / Pornparn Kongpracha / Satomi Moriyama / Deryck J Mills / Werner Kühlbrandt / Shushi Nagamori /
PubMed AbstractCystinuria is a genetic disorder characterized by overexcretion of dibasic amino acids and cystine, causing recurrent kidney stones and kidney failure. Mutations of the regulatory glycoprotein rBAT ...Cystinuria is a genetic disorder characterized by overexcretion of dibasic amino acids and cystine, causing recurrent kidney stones and kidney failure. Mutations of the regulatory glycoprotein rBAT and the amino acid transporter bAT, which constitute system b, are linked to type I and non-type I cystinuria respectively and they exhibit distinct phenotypes due to protein trafficking defects or catalytic inactivation. Here, using electron cryo-microscopy and biochemistry, we discover that Ca mediates higher-order assembly of system b. Ca stabilizes the interface between two rBAT molecules, leading to super-dimerization of bAT-rBAT, which in turn facilitates N-glycan maturation and protein trafficking. A cystinuria mutant T216M and mutations of the Ca site of rBAT cause the loss of higher-order assemblies, resulting in protein trapping at the ER and the loss of function. These results provide the molecular basis of system b biogenesis and type I cystinuria and serve as a guide to develop new therapeutic strategies against it. More broadly, our findings reveal an unprecedented link between transporter oligomeric assembly and protein-trafficking diseases.
External linksNat Commun / PubMed:35577790 / PubMed Central
MethodsEM (single particle)
Resolution2.68 - 3.05 Å
Structure data

12296

7nf6

EMDB-12296, PDB-7nf6:
Ovine b0,+AT-rBAT heterodimer
Method: EM (single particle) / Resolution: 3.05 Å

12297

7nf7

EMDB-12297: Ovine rBAT ectodomain homodimer, C2-symmetry
PDB-7nf7: Ovine rBAT ectodomain homodimer, asymmetric unit
Method: EM (single particle) / Resolution: 2.68 Å

12298

7nf8

EMDB-12298: Ovine (b0,+AT-rBAT)2 hetero-tetramer, consensus refinement, C2-symmetry
PDB-7nf8: Ovine (b0,+AT-rBAT)2 hetero-tetramer, asymmetric unit, rigid-body fitted
Method: EM (single particle) / Resolution: 2.83 Å

Chemicals

ChemComp-CLR:
CHOLESTEROL / Cholesterol

ChemComp-LBN:
1-palmitoyl-2-oleoyl-sn-glycero-3-phosphocholine / phospholipid*YM / POPC

ChemComp-CA:
Unknown entry

ChemComp-NAG:
2-acetamido-2-deoxy-beta-D-glucopyranose / N-Acetylglucosamine

Source
  • ovis aries (sheep)
KeywordsTRANSLOCASE / Transporter / Cystinuria / Complex / Ectodomain

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