LMAN1_HUMAN » Protein ERGIC-53

LMAN1_HUMAN » Protein ERGIC-53
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Topology in Endoplasmic reticulum membrane
Topologylumenal side
cytoplasmic side
LMAN1_HUMAN » Protein ERGIC-53 » ER-Golgi intermediate compartment 53 kDa protein;Gp58;Intracellular mannose-specific lectin MR60;Lectin mannose-binding 1;
Hydrophobic Thickness 30.8 ± 1.4 Å
Tilt Angle 0 ± 0°
ΔGtransfer -36.0 kcal/mol
ΔGfold -17.6 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology Out
TM Segments 477-499 (473-506)
Pathways

Protein processing in endoplasmic reticulum (KEGG)

PDB 4gkx (31-270), 4gky (31-270), 3lcp (A/B=32-277), 3wht (A=31-269), 3whu (A=31-269), 3wnx (A=31-269), 3a4u (A=31-285)
OPM none
Complexes none
Interactions

A4, Complex: A4:LMAN1, PubMed

CALX, Complex: LMAN1:CALX, PubMed

PTN2, Complex: PTN2:LMAN1, PubMed

TMED2, Complex: LMAN1:TMED2, PubMed

TMED5, Complex: TMED5:LMAN1

Domains

AA: 44-269, PDBID: 3A4U, Subunit A, Seq Identity:100%, Legume-like lectin family

UniProt annotation for LMAN1_HUMAN » Protein ERGIC-53
FUNCTION: Mannose-specific lectin. May recognize sugar residues of glycoproteins, glycolipids, or glycosylphosphatidyl inositol anchors and may be involved in the sorting or recycling of proteins, lipids, or both. The LMAN1-MCFD2 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins.

SUBUNIT: Exists both as a covalent disulfide-linked homohexamer, and a complex of three disulfide-linked dimers non-covalently kept together. Interacts with MCFD2. May interact with TMEM115.

TISSUE SPECIFICITY: Ubiquitous.

DOMAIN: The FF ER export motif at the C-terminus is not sufficient to support endoplasmic reticulum exit, and needs assistance of Gln-501 for proper recognition of COPII coat components.

DISEASE: Factor V and factor VIII combined deficiency 1 (F5F8D1) OMIM: A blood coagulation disorder characterized by bleeding symptoms similar to those in hemophilia or parahemophilia, that are caused by single deficiency of FV or FVIII, respectively. The most common symptoms are epistaxis, menorrhagia, and excessive bleeding during or after trauma. Plasma levels of coagulation factors V and VIII are in the range of 5 to 30% of normal. mutations affecting the gene represented in this entry.

UniProt features for LMAN1_HUMAN » Protein ERGIC-53
SIGNAL 1 30
CHAIN 31 510 Protein ERGIC-53.
DOMAIN 44 267 L-type lectin-like.
REGION 251 253 Carbohydrate binding (By similarity).
MOTIF 509 510 ER export motif.
SITE 501 501 Required for ER export.
DISULFID 190 230
DISULFID 466 466 Interchain.
DISULFID 475 475 Interchain.
Amino Acid Sequence for LMAN1_HUMAN » Protein ERGIC-53
MAGSRQRGLR ARVRPLFCAL LLSLGRFVRG DGVGGDPAVA LPHRRFEYKY SFKGPHLVQS DGTVPFWAHA GNAIPSSDQI RVAPSLKSQR GSVWTKTKAA FENWEVEVTF RVTGRGRIGA DGLAIWYAEN QGLEGPVFGS ADLWNGVGIF FDSFDNDGKK NNPAIVIIGN NGQIHYDHQN DGASQALASC QRDFRNKPYP VRAKITYYQN TLTVMINNGF TPDKNDYEFC AKVENMIIPA QGHFGISAAT GGLADDHDVL SFLTFQLTEP GKEPPTPDKE ISEKEKEKYQ EEFEHFQQEL DKKKEEFQKG HPDLQGQPAE EIFESVGDRE LRQVFEGQNR IHLEIKQLNR QLDMILDEQR RYVSSLTEEI SKRGAGMPGQ HGQITQQELD TVVKTQHEIL RQVNEMKNSM SETVRLVSGM QHPGSAGGVY ETTQHFIDIK EHLHIVKRDI DNLVQRNMPS NEKPKCPELP PFPSCLSTVH FIIFVVVQTV LFIGYIMYRS QQEAAAKKFF