Minor histocompatibility antigen H13

Details

Name
Minor histocompatibility antigen H13
Synonyms
  • 3.4.23.-
  • H13
  • hIMP1
  • IMP-1
  • IMP1
  • IMPAS-1
  • Intramembrane protease 1
  • Presenilin-like protein 3
  • PSL3
  • Signal peptide peptidase
  • SPP
Gene Name
HM13
Organism
Humans
Amino acid sequence
>lcl|BSEQ0049720|Minor histocompatibility antigen H13
MDSALSDPHNGSAEAGGPTNSTTRPPSTPEGIALAYGSLLLMALLPIFFGALRSVRCARG
KNASDMPETITSRDAARFPIIASCTLLGLYLFFKIFSQEYINLLLSMYFFVLGILALSHT
ISPFMNKFFPASFPNRQYQLLFTQGSGENKEEIINYEFDTKDLVCLGLSSIVGVWYLLRK
HWIANNLFGLAFSLNGVELLHLNNVSTGCILLGGLFIYDVFWVFGTNVMVTVAKSFEAPI
KLVFPQDLLEKGLEANNFAMLGLGDVVIPGIFIALLLRFDISLKKNTHTYFYTSFAAYIF
GLGLTIFIMHIFKHAQPALLYLVPACIGFPVLVALAKGEVTEMFSYEESNPKDPAAVTES
KEGTEASASKGLEKKEK
Number of residues
377
Molecular Weight
41487.86
Theoretical pI
Not Available
GO Classification
Functions
aspartic endopeptidase activity, intramembrane cleaving / peptidase activity / protein homodimerization activity / ubiquitin protein ligase binding
Processes
membrane protein proteolysis / membrane protein proteolysis involved in retrograde protein transport, ER to cytosol / protein homotetramerization
Components
cell surface / Derlin-1 retrotranslocation complex / endoplasmic reticulum / endoplasmic reticulum membrane / Golgi-associated vesicle membrane / integral component of cytoplasmic side of endoplasmic reticulum membrane / integral component of lumenal side of endoplasmic reticulum membrane / lysosomal membrane / membrane / plasma membrane / rough endoplasmic reticulum
General Function
Catalyzes intramembrane proteolysis of some signal peptides after they have been cleaved from a preprotein, resulting in the release of the fragment from the ER membrane into the cytoplasm. Required to generate lymphocyte cell surface (HLA-E) epitopes derived from MHC class I signal peptides (PubMed:11714810). May be necessary for the removal of the signal peptide that remains attached to the hepatitis C virus core protein after the initial proteolytic processing of the polyprotein (PubMed:12145199). Involved in the intramembrane cleavage of the integral membrane protein PSEN1 (PubMed:12077416, PubMed:11714810, PubMed:14741365). Cleaves the integral membrane protein XBP1 isoform 1 in a DERL1/RNF139-dependent manner (PubMed:25239945). May play a role in graft rejection (By similarity).
Specific Function
Aspartic endopeptidase activity, intramembrane cleaving
Pfam Domain Function
Transmembrane Regions
32-52 78-98 101-121 158-178 182-202 210-230 257-277 291-311 315-335
Cellular Location
Endoplasmic reticulum membrane
Gene sequence
>lcl|BSEQ0049721|Minor histocompatibility antigen H13 (HM13)
ATGGACTCGGCCCTCAGCGATCCGCATAACGGCAGTGCCGAGGCAGGCGGCCCCACCAAC
AGCACTACGCGGCCGCCTTCCACGCCCGAGGGCATCGCGCTGGCCTACGGCAGCCTCCTG
CTCATGGCGCTGCTGCCCATCTTCTTCGGCGCCCTGCGCTCCGTACGCTGCGCCCGCGGC
AAGAATGCTTCAGACATGCCTGAAACAATCACCAGCCGGGATGCCGCCCGCTTCCCCATC
ATCGCCAGCTGCACACTCTTGGGGCTCTACCTCTTTTTCAAAATATTCTCCCAGGAGTAC
ATCAACCTCCTGCTGTCCATGTATTTCTTCGTGCTGGGAATCCTGGCCCTGTCCCACACC
ATCAGCCCCTTCATGAATAAGTTTTTTCCAGCCAGCTTTCCAAATCGACAGTACCAGCTG
CTCTTCACACAGGGTTCTGGGGAAAACAAGGAAGAGATCATCAATTATGAATTTGACACC
AAGGACCTGGTGTGCCTGGGCCTGAGCAGCATCGTTGGCGTCTGGTACCTGCTGAGGAAG
CACTGGATTGCCAACAACCTTTTTGGCCTGGCCTTCTCCCTTAATGGAGTAGAGCTCCTG
CACCTCAACAATGTCAGCACTGGCTGCATCCTGCTGGGCGGACTCTTCATCTACGATGTC
TTCTGGGTATTTGGCACCAATGTGATGGTGACAGTGGCCAAGTCCTTCGAGGCACCAATA
AAATTGGTGTTTCCCCAGGATCTGCTGGAGAAAGGCCTCGAAGCAAACAACTTTGCCATG
CTGGGACTTGGAGATGTCGTCATTCCAGGGATCTTCATTGCCTTGCTGCTGCGCTTTGAC
ATCAGCTTGAAGAAGAATACCCACACCTACTTCTACACCAGCTTTGCAGCCTACATCTTC
GGCCTGGGCCTTACCATCTTCATCATGCACATCTTCAAGCATGCTCAGCCTGCCCTCCTA
TACCTGGTCCCCGCCTGCATCGGTTTTCCTGTCCTGGTGGCGCTGGCCAAGGGAGAAGTG
ACAGAGATGTTCAGTTATGAGGAGTCAAATCCTAAGGATCCAGCGGCAGTGACAGAATCC
AAAGAGGGAACAGAGGCATCAGCATCGAAGGGGCTGGAGAAGAAAGAGAAATGA
Chromosome Location
20
Locus
20q11.21
External Identifiers
ResourceLink
UniProtKB IDQ8TCT9
UniProtKB Entry NameHM13_HUMAN
HGNC IDHGNC:16435
General References
  1. Weihofen A, Binns K, Lemberg MK, Ashman K, Martoglio B: Identification of signal peptide peptidase, a presenilin-type aspartic protease. Science. 2002 Jun 21;296(5576):2215-8. [Article]
  2. Urny J, Hermans-Borgmeyer I, Gercken G, Schaller HC: Expression of the presenilin-like signal peptide peptidase (SPP) in mouse adult brain and during development. Gene Expr Patterns. 2003 Oct;3(5):685-91. [Article]
  3. Grigorenko AP, Moliaka YK, Korovaitseva GI, Rogaev EI: Novel class of polytopic proteins with domains associated with putative protease activity. Biochemistry (Mosc). 2002 Jul;67(7):826-35. [Article]
  4. Urny J, Hermans-Borgmeyer I, Schaller HC: Cell-surface expression of a new splice variant of the mouse signal peptide peptidase. Biochim Biophys Acta. 2006 Mar-Apr;1759(3-4):159-65. Epub 2006 Mar 31. [Article]
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  7. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [Article]
  8. Lemberg MK, Bland FA, Weihofen A, Braud VM, Martoglio B: Intramembrane proteolysis of signal peptides: an essential step in the generation of HLA-E epitopes. J Immunol. 2001 Dec 1;167(11):6441-6. [Article]
  9. McLauchlan J, Lemberg MK, Hope G, Martoglio B: Intramembrane proteolysis promotes trafficking of hepatitis C virus core protein to lipid droplets. EMBO J. 2002 Aug 1;21(15):3980-8. [Article]
  10. Moliaka YK, Grigorenko A, Madera D, Rogaev EI: Impas 1 possesses endoproteolytic activity against multipass membrane protein substrate cleaving the presenilin 1 holoprotein. FEBS Lett. 2004 Jan 16;557(1-3):185-92. [Article]
  11. Friedmann E, Lemberg MK, Weihofen A, Dev KK, Dengler U, Rovelli G, Martoglio B: Consensus analysis of signal peptide peptidase and homologous human aspartic proteases reveals opposite topology of catalytic domains compared with presenilins. J Biol Chem. 2004 Dec 3;279(49):50790-8. Epub 2004 Sep 21. [Article]
  12. Krawitz P, Haffner C, Fluhrer R, Steiner H, Schmid B, Haass C: Differential localization and identification of a critical aspartate suggest non-redundant proteolytic functions of the presenilin homologues SPPL2b and SPPL3. J Biol Chem. 2005 Nov 25;280(47):39515-23. Epub 2005 Jul 5. [Article]
  13. Stagg HR, Thomas M, van den Boomen D, Wiertz EJ, Drabkin HA, Gemmill RM, Lehner PJ: The TRC8 E3 ligase ubiquitinates MHC class I molecules before dislocation from the ER. J Cell Biol. 2009 Sep 7;186(5):685-92. doi: 10.1083/jcb.200906110. Epub 2009 Aug 31. [Article]
  14. Chen R, Jiang X, Sun D, Han G, Wang F, Ye M, Wang L, Zou H: Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry. J Proteome Res. 2009 Feb;8(2):651-61. doi: 10.1021/pr8008012. [Article]
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  16. Chen CY, Malchus NS, Hehn B, Stelzer W, Avci D, Langosch D, Lemberg MK: Signal peptide peptidase functions in ERAD to cleave the unfolded protein response regulator XBP1u. EMBO J. 2014 Nov 3;33(21):2492-506. doi: 10.15252/embj.201488208. Epub 2014 Sep 19. [Article]
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB00277TheophyllineapprovedunknownDetails