+
Open data
-
Basic information
Entry | Database: SASBDB / ID: SASDDW4 |
---|---|
![]() | Procollagen lysyl hydroxylase LH3 measured using SEC-SAXS
|
Function / homology | ![]() procollagen galactosyltransferase / procollagen glucosyltransferase / peptidyl-lysine hydroxylation / procollagen glucosyltransferase activity / hydroxylysine biosynthetic process / procollagen galactosyltransferase activity / procollagen-lysine 5-dioxygenase / procollagen-lysine 5-dioxygenase activity / basement membrane assembly / epidermis morphogenesis ...procollagen galactosyltransferase / procollagen glucosyltransferase / peptidyl-lysine hydroxylation / procollagen glucosyltransferase activity / hydroxylysine biosynthetic process / procollagen galactosyltransferase activity / procollagen-lysine 5-dioxygenase / procollagen-lysine 5-dioxygenase activity / basement membrane assembly / epidermis morphogenesis / Collagen biosynthesis and modifying enzymes / collagen metabolic process / endothelial cell morphogenesis / protein O-linked glycosylation / L-ascorbic acid binding / collagen fibril organization / neural tube development / lung morphogenesis / small molecule binding / rough endoplasmic reticulum / protein localization / trans-Golgi network / vasodilation / collagen-containing extracellular matrix / in utero embryonic development / iron ion binding / endoplasmic reticulum lumen / endoplasmic reticulum membrane / Golgi apparatus / endoplasmic reticulum / extracellular space / extracellular exosome / metal ion binding Similarity search - Function |
Biological species | ![]() |
![]() | ![]() Title: Molecular architecture of the multifunctional collagen lysyl hydroxylase and glycosyltransferase LH3. Authors: Luigi Scietti / Antonella Chiapparino / Francesca De Giorgi / Marco Fumagalli / Lela Khoriauli / Solomon Nergadze / Shibom Basu / Vincent Olieric / Lucia Cucca / Blerida Banushi / Antonella ...Authors: Luigi Scietti / Antonella Chiapparino / Francesca De Giorgi / Marco Fumagalli / Lela Khoriauli / Solomon Nergadze / Shibom Basu / Vincent Olieric / Lucia Cucca / Blerida Banushi / Antonella Profumo / Elena Giulotto / Paul Gissen / Federico Forneris / ![]() ![]() ![]() ![]() Abstract: Lysyl hydroxylases catalyze hydroxylation of collagen lysines, and sustain essential roles in extracellular matrix (ECM) maturation and remodeling. Malfunctions in these enzymes cause severe ...Lysyl hydroxylases catalyze hydroxylation of collagen lysines, and sustain essential roles in extracellular matrix (ECM) maturation and remodeling. Malfunctions in these enzymes cause severe connective tissue disorders. Human lysyl hydroxylase 3 (LH3/PLOD3) bears multiple enzymatic activities, as it catalyzes collagen lysine hydroxylation and also their subsequent glycosylation. Our understanding of LH3 functions is currently hampered by lack of molecular structure information. Here, we present high resolution crystal structures of full-length human LH3 in complex with cofactors and donor substrates. The elongated homodimeric LH3 architecture shows two distinct catalytic sites at the N- and C-terminal boundaries of each monomer, separated by an accessory domain. The glycosyltransferase domain displays distinguishing features compared to other known glycosyltransferases. Known disease-related mutations map in close proximity to the catalytic sites. Collectively, our results provide a structural framework characterizing the multiple functions of LH3, and the molecular mechanisms of collagen-related diseases involving human lysyl hydroxylases. |
![]() |
|
-
Structure visualization
Structure viewer | Molecule: ![]() ![]() |
---|
-
Downloads & links
-Data source
SASBDB page | ![]() |
---|
-Related structure data
Related structure data | ![]() 6fxkC ![]() 6fxmC ![]() 6fxrC ![]() 6fxtC ![]() 6fxxC ![]() 6fxyC C: citing same article ( |
---|---|
Similar structure data |
-
External links
Related items in Molecule of the Month |
---|
-Models
Model #1862 | ![]() Type: dummy / Radius of dummy atoms: 1.90 A / Chi-square value: 1.85 ![]() |
---|
-
Sample
![]() | Name: Procollagen lysyl hydroxylase LH3 measured using SEC-SAXS Specimen concentration: 3.5 mg/ml |
---|---|
Buffer | Name: 25 mM HEPES, 200 mM NaCl / pH: 8 |
Entity #1000 | Name: LH3 / Type: protein / Description: Procollagen lysyl hydroxylase LH3 / Formula weight: 90 / Num. of mol.: 2 / Source: Homo sapiens / References: UniProt: O60568 Sequence: SSDRPRGRDP VNPEKLLVIT VATAETEGYL RFLRSAEFFN YTVRTLGLGE EWRGGDVART VGGGQKVRWL KKEMEKYADR EDMIIMFVDS YDVILAGSPT ELLKKFVQSG SRLLFSAESF CWPEWGLAEQ YPEVGTGKRF LNSGGFIGFA TTIHQIVRQW KYKDDDDDQL ...Sequence: SSDRPRGRDP VNPEKLLVIT VATAETEGYL RFLRSAEFFN YTVRTLGLGE EWRGGDVART VGGGQKVRWL KKEMEKYADR EDMIIMFVDS YDVILAGSPT ELLKKFVQSG SRLLFSAESF CWPEWGLAEQ YPEVGTGKRF LNSGGFIGFA TTIHQIVRQW KYKDDDDDQL FYTRLYLDPG LREKLSLNLD HKSRIFQNLN GALDEVVLKF DRNRVRIRNV AYDTLPIVVH GNGPTKLQLN YLGNYVPNGW TPEGGCGFCN QDRRTLPGGQ PPPRVFLAVF VEQPTPFLPR FLQRLLLLDY PPDRVTLFLH NNEVFHEPHI ADSWPQLQDH FSAVKLVGPE EALSPGEARD MAMDLCRQDP ECEFYFSLDA DAVLTNLQTL RILIEENRKV IAPMLSRHGK LWSNFWGALS PDEYYARSED YVELVQRKRV GVWNVPYISQ AYVIRGDTLR MELPQRDVFS GSDTDPDMAF CKSFRDKGIF LHLSNQHEFG RLLATSRYDT EHLHPDLWQI FDNPVDWKEQ YIHENYSRAL EGEGIVEQPC PDVYWFPLLS EQMCDELVAE MEHYGQWSGG RHEDSRLAGG YENVPTVDIH MKQVGYEDQW LQLLRTYVGP MTESLFPGYH TKARAVMNFV VRYRPDEQPS LRPHHDSSTF TLNVALNHKG LDYEGGGCRF LRYDCVISSP RKGWALLHPG RLTHYHEGLP TTWGTRYIMV SFVDPAAA |
-Experimental information
Beam | Instrument name: ESRF BM29 / City: Grenoble / 国: France ![]() | |||||||||||||||||||||||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Detector | Name: Pilatus 1M / Pixsize x: 172 mm | |||||||||||||||||||||||||||||||||||||||
Scan | Measurement date: Feb 28, 2018 / Storage temperature: 20 °C / Cell temperature: 20 °C / Exposure time: 1 sec. / Number of frames: 75 / Unit: 1/nm /
| |||||||||||||||||||||||||||||||||||||||
Distance distribution function P(R) |
| |||||||||||||||||||||||||||||||||||||||
Result | Comments: The sample was injected at a load concentration of 3.5 mg/mL. The SEC column used was a Superdex 200 PC 3.2/300 Increase (GE Healthcare) at a flow rate 0.075 mL/min.
|