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TitleStructural basis of dual Ca/pH regulation of the endolysosomal TRPML1 channel.
Journal, issue, pagesNat Struct Mol Biol, Vol. 24, Issue 3, Page 205-213, Year 2017
Publish dateJan 23, 2017
AuthorsMinghui Li / Wei K Zhang / Nicole M Benvin / Xiaoyuan Zhou / Deyuan Su / Huan Li / Shu Wang / Ioannis E Michailidis / Liang Tong / Xueming Li / Jian Yang /
PubMed AbstractThe activities of organellar ion channels are often regulated by Ca and H, which are present in high concentrations in many organelles. Here we report a structural element critical for dual Ca/pH ...The activities of organellar ion channels are often regulated by Ca and H, which are present in high concentrations in many organelles. Here we report a structural element critical for dual Ca/pH regulation of TRPML1, a Ca-release channel crucial for endolysosomal function. TRPML1 mutations cause mucolipidosis type IV (MLIV), a severe lysosomal storage disorder characterized by neurodegeneration, mental retardation and blindness. We obtained crystal structures of the 213-residue luminal domain of human TRPML1 containing three missense MLIV-causing mutations. This domain forms a tetramer with a highly electronegative central pore formed by a novel luminal pore loop. Cysteine cross-linking and cryo-EM analyses confirmed that this architecture occurs in the full-length channel. Structure-function studies demonstrated that Ca and H interact with the luminal pore and exert physiologically important regulation. The MLIV-causing mutations disrupt the luminal-domain structure and cause TRPML1 mislocalization. Our study reveals the structural underpinnings of TRPML1's regulation, assembly and pathogenesis.
External linksNat Struct Mol Biol / PubMed:28112729 / PubMed Central
MethodsEM (single particle) / X-ray diffraction
Resolution2.3 - 8.12 Å
Structure data

EMDB-6669:
The endolysosomal Ca2+ channel TRPML1
Method: EM (single particle) / Resolution: 8.12 Å

EMDB-6670:
The endolysosomal Ca2+ channel TRPML1
Method: EM (single particle) / Resolution: 5.28 Å

PDB-5tja:
I-II linker of TRPML1 channel at pH 6
Method: X-RAY DIFFRACTION / Resolution: 2.3 Å

PDB-5tjb:
I-II linker of TRPML1 channel at pH 4.5
Method: X-RAY DIFFRACTION / Resolution: 2.4 Å

PDB-5tjc:
I-II linker of TRPML1 channel at pH 7.5
Method: X-RAY DIFFRACTION / Resolution: 2.4 Å

Chemicals

ChemComp-HOH:
WATER / Water

Source
  • Caenorhabditis elegans (invertebrata)
  • homo sapiens (human)
KeywordsTRANSPORT PROTEIN / endolysosomal lumen / tetramer / calcium and pH regulation

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