KE04

Summary

Gene Symbol: KE04
Description: ER lipid raft associated 1
Alias: C10orf69, Erlin-1, KE04, KEO4, SPFH1, SPG62, erlin-1, Band_7 23-211 Keo4 (Interim) similar to C.elegans protein C42C1.9, SPFH domain family, member 1, SPFH domain-containing protein 1, endoplasmic reticulum lipid raft-associated protein 1, stomatin-prohibitin-flotillin-HflC/K domain-containing protein 1
Species: human

Top Publications

  1. ncbi Erlin-1 and erlin-2 are novel members of the prohibitin family of proteins that define lipid-raft-like domains of the ER
    Duncan T Browman
    Southern Alberta Cancer Research Institute, Departments of Oncology and Biochemistry and Molecular Biology, University of Calgary, Calgary, Alberta, T2N 4N1, Canada
    J Cell Sci 119:3149-60. 2006
  2. pmc An endoplasmic reticulum (ER) membrane complex composed of SPFH1 and SPFH2 mediates the ER-associated degradation of inositol 1,4,5-trisphosphate receptors
    Margaret M P Pearce
    Departments of Pharmacology and Biochemistry and Molecular Biology, SUNY Upstate Medical University, Syracuse, New York 13210, USA
    J Biol Chem 284:10433-45. 2009
  3. pmc Genomic structural variations for cardiovascular and metabolic comorbidity
    Maria S Nazarenko
    Laboratory of Population Genetics, Research Institute of Medical Genetics, Tomsk National Research Medical Center, Russian Academy of Sciences, Tomsk, Russia
    Sci Rep 7:41268. 2017
  4. pmc Mechanistically linked serum miRNAs distinguish between drug induced and fatty liver disease of different grades
    Zhichao Liu
    Division of Bioinformatics and Biostatistics, National Center for Toxicological Research, U S Food and Drug Administration, Jefferson, Arkansas, USA
    Sci Rep 6:23709. 2016
  5. doi A genome wide association study (GWAS) providing evidence of an association between common genetic variants and late radiotherapy toxicity
    Gillian C Barnett
    Centre for Cancer Genetic Epidemiology, University of Cambridge, Strangeways Research Laboratory, UK University of Cambridge, Department of Oncology, Oncology Centre, Cambridge University Hospitals NHS Foundation Trust, UK Electronic address
    Radiother Oncol 111:178-85. 2014
  6. ncbi Association of polymorphisms in GCKR and TRIB1 with nonalcoholic fatty liver disease and metabolic syndrome traits
    Aya Kitamoto
    Medical Research Support Center, Pharmacogenomics, Kyoto University Graduate School of Medicine, Kyoto 606 8501, Japan
    Endocr J 61:683-9. 2014
  7. pmc Exome sequencing links corticospinal motor neuron disease to common neurodegenerative disorders
    Gaia Novarino
    Howard Hughes Medical Institute, University of California, San Diego, La Jolla, CA 92093, USA
    Science 343:506-11. 2014
  8. pmc Erlins restrict SREBP activation in the ER and regulate cellular cholesterol homeostasis
    Michael D Huber
    Department of Cell and Molecular Biology, The Scripps Research Institute, La Jolla, CA 92037
    J Cell Biol 203:427-36. 2013
  9. pmc Endoplasmic reticulum factor ERLIN2 regulates cytosolic lipid content in cancer cells
    Guohui Wang
    Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, MI 48201, USA
    Biochem J 446:415-25. 2012
  10. doi Detection of dissolved organic matter in saline-alkali soils using synchronous fluorescence spectroscopy and principal component analysis
    Xu jing Guo
    Key Laboratory of Development and Application of Rural Renewable Energy, Biogas Scientific Research Institute of the Ministry of Agriculture, Chengdu 610041, China
    Spectrochim Acta A Mol Biomol Spectrosc 104:280-6. 2013

Research Grants

Scientific Experts

  • Gillian C Barnett
  • Mary F Feitosa
  • Richard Wojcikiewicz
  • Markus Hinderhofer
  • LARRY R GERACE
  • Yuan Wang
  • Margaret M P Pearce
  • Maria S Nazarenko
  • Zhichao Liu
  • Duncan T Browman
  • Gaia Novarino
  • Aya Kitamoto
  • Xu jing Guo
  • Michael D Huber
  • Ebru Boslem
  • Guohui Wang
  • Deepa Pednekar
  • Justine P Lu
  • Tetsuro Yoshida
  • Danielle A Sliter
  • Maja B Hoegg
  • Xin Yuan
  • Stephen M Robbins
  • Mary E Resek
  • Maria V Golubenko
  • Nikolay A Skryabin
  • Iuliia A Koroleva
  • Valery P Puzyrev
  • Olga L Barbarash
  • Anton V Markov
  • Igor N Lebedev
  • Anton N Kazancev
  • Aleksei A Sleptcov
  • Jurgen Borlak
  • Yuping Wang
  • Weida Tong
  • Joseph G Gleeson
  • P S Bindu
  • Massimo Mascaro
  • Ali G Fenstermaker
  • Mahmoud Y Issa
  • Faezeh Mojahedi
  • Ahmed Bouhouche
  • Jumana Y Al-Aama
  • Jennifer L Silhavy
  • Takato Ueno
  • Takuya Kitamoto
  • Amira Masri
  • Ariana Karminejad
  • Hisham Megahed
  • Hulya Kayserili
  • Ghada M H Abdel-Salam
  • Masato Yoneda
  • Bita Bozorgmehri
  • Akihiro Sekine
  • Stacy B Gabriel
  • Kazuwa Nakao
  • Alexis Brice
  • Başak Rosti
  • Sylvain Hanein
  • Atsushi Nakajima
  • Mostafa Abdellateef
  • Hideyuki Hyogo
  • Naima Bouslam
  • Majdi Kara
  • Hidenori Ochi
  • Seiho Mizusawa
  • Alexandra Durr
  • Murat Gunel
  • Trey Ideker
  • Alice A Koh
  • Rasim O Rosti
  • Tawfeg Ben-Omran
  • Andrew D Heiberg
  • Yuji Ogawa
  • Jana Schroth
  • Laure Raymond
  • Matloob Azam
  • Laila Selim
  • Keith K Vaux
  • Nabil Shehata
  • Ali Benomar
  • Maha S Zaki
  • Emily G Spencer
  • Matan Hofree
  • Naiara Akizu
  • Kaya Bilguvar
  • Lobna Mansour
  • Giovanni Stevanin
  • Bulent Kara

Detail Information

Publications21

  1. ncbi Erlin-1 and erlin-2 are novel members of the prohibitin family of proteins that define lipid-raft-like domains of the ER
    Duncan T Browman
    Southern Alberta Cancer Research Institute, Departments of Oncology and Biochemistry and Molecular Biology, University of Calgary, Calgary, Alberta, T2N 4N1, Canada
    J Cell Sci 119:3149-60. 2006
    ..Interestingly, the ER contains relatively low levels of cholesterol and sphingolipids compared with other organelles. Thus, our data support the existence of lipid-raft-like domains within the membranes of the ER...
  2. pmc An endoplasmic reticulum (ER) membrane complex composed of SPFH1 and SPFH2 mediates the ER-associated degradation of inositol 1,4,5-trisphosphate receptors
    Margaret M P Pearce
    Departments of Pharmacology and Biochemistry and Molecular Biology, SUNY Upstate Medical University, Syracuse, New York 13210, USA
    J Biol Chem 284:10433-45. 2009
    ..Here we report that the ER membrane protein SPFH1 and its homolog SPFH2 form a heteromeric approximately 2 MDa complex that binds to IP(3)R tetramers immediately ..
  3. pmc Genomic structural variations for cardiovascular and metabolic comorbidity
    Maria S Nazarenko
    Laboratory of Population Genetics, Research Institute of Medical Genetics, Tomsk National Research Medical Center, Russian Academy of Sciences, Tomsk, Russia
    Sci Rep 7:41268. 2017
    ..31 (ERLIN1) and 12q24.11 (UNG, ACACB) genomic regions in patients with coronary artery disease and metabolic comorbidity. Analysis of DNA extracted from blood indicated a possible somatic origin for these CNVs...
  4. pmc Mechanistically linked serum miRNAs distinguish between drug induced and fatty liver disease of different grades
    Zhichao Liu
    Division of Bioinformatics and Biostatistics, National Center for Toxicological Research, U S Food and Drug Administration, Jefferson, Arkansas, USA
    Sci Rep 6:23709. 2016
    ..Altogether, serum miRNAs informed on the molecular pathophysiology of NAFLD and permitted differentiation between DIS and NAFLD of different grades. ..
  5. doi A genome wide association study (GWAS) providing evidence of an association between common genetic variants and late radiotherapy toxicity
    Gillian C Barnett
    Centre for Cancer Genetic Epidemiology, University of Cambridge, Strangeways Research Laboratory, UK University of Cambridge, Department of Oncology, Oncology Centre, Cambridge University Hospitals NHS Foundation Trust, UK Electronic address
    Radiother Oncol 111:178-85. 2014
    ..This study was designed to identify common single nucleotide polymorphisms (SNPs) associated with toxicity 2years after radiotherapy...
  6. ncbi Association of polymorphisms in GCKR and TRIB1 with nonalcoholic fatty liver disease and metabolic syndrome traits
    Aya Kitamoto
    Medical Research Support Center, Pharmacogenomics, Kyoto University Graduate School of Medicine, Kyoto 606 8501, Japan
    Endocr J 61:683-9. 2014
    ..Our data suggest variations in GCKR and TRIB1 are involved in the development of nonalcoholic fatty liver disease...
  7. pmc Exome sequencing links corticospinal motor neuron disease to common neurodegenerative disorders
    Gaia Novarino
    Howard Hughes Medical Institute, University of California, San Diego, La Jolla, CA 92093, USA
    Science 343:506-11. 2014
    ..Our analysis links HSP to other neurodegenerative disorders and can facilitate gene discovery and mechanistic understanding of disease. ..
  8. pmc Erlins restrict SREBP activation in the ER and regulate cellular cholesterol homeostasis
    Michael D Huber
    Department of Cell and Molecular Biology, The Scripps Research Institute, La Jolla, CA 92037
    J Cell Biol 203:427-36. 2013
    ..We speculate that erlins promote stability of the SREBP-Scap-Insig complex and may contribute to the highly cooperative control of this system. ..
  9. pmc Endoplasmic reticulum factor ERLIN2 regulates cytosolic lipid content in cancer cells
    Guohui Wang
    Center for Molecular Medicine and Genetics, Wayne State University School of Medicine, Detroit, MI 48201, USA
    Biochem J 446:415-25. 2012
    ..The identification of ERLIN2 as a regulator of cytosolic lipid content in cancer cells has important implications for understanding the molecular basis of tumorigenesis and the treatment of cancer...
  10. doi Detection of dissolved organic matter in saline-alkali soils using synchronous fluorescence spectroscopy and principal component analysis
    Xu jing Guo
    Key Laboratory of Development and Application of Rural Renewable Energy, Biogas Scientific Research Institute of the Ministry of Agriculture, Chengdu 610041, China
    Spectrochim Acta A Mol Biomol Spectrosc 104:280-6. 2013
    ..9, PC2<0.3), including soils Ke02, Ke03, Ke04, Rh02, Rh03, Rh04, Sa04 and Sc04...
  11. pmc The ERLIN1-CHUK-CWF19L1 gene cluster influences liver fat deposition and hepatic inflammation in the NHLBI Family Heart Study
    Mary F Feitosa
    Department of Genetics, Washington University School of Medicine, St Louis, MO 63108 2212, USA
    Atherosclerosis 228:175-80. 2013
    ..We sought to identify common genetic variants contributing to NAFLD, using CT measured fatty liver (FL), and alanine aminotransferase levels (ALT), as a biochemical marker of hepatic inflammation...
  12. pmc Alteration of endoplasmic reticulum lipid rafts contributes to lipotoxicity in pancreatic β-cells
    Ebru Boslem
    From the Diabetes and Obesity Program, Garvan Institute of Medical Research, 384 Victoria St, Darlinghurst, New South Wales 2010, Australia and
    J Biol Chem 288:26569-82. 2013
    ..Our results suggest that loss of SM in the ER is a key event for initiating β-cell lipotoxicity, which leads to disruption of ER lipid rafts, perturbation of protein trafficking, and initiation of ER stress. ..
  13. pmc RNF170 protein, an endoplasmic reticulum membrane ubiquitin ligase, mediates inositol 1,4,5-trisphosphate receptor ubiquitination and degradation
    Justine P Lu
    Department of Pharmacology, State University of New York Upstate Medical University, Syracuse, New York 13210, USA
    J Biol Chem 286:24426-33. 2011
    ..A substantial proportion of RNF170 is constitutively associated with the erlin1/2 (SPFH1/2) complex, which has been shown previously to bind to IP(3) receptors immediately after their activation...
  14. pmc Clustered hydrophobic amino acids in amphipathic helices mediate erlin1/2 complex assembly
    Deepa Pednekar
    Department of Pharmacology, SUNY Upstate Medical University, 750 E Adams St, Syracuse, NY 13210, USA
    Biochem Biophys Res Commun 415:135-40. 2011
    ..Overall, we conclude that the ∼2 MDa erlin1/2 complex is composed of an assemblage of lower-order hetero-oligomers, probably heterotrimers, linked together by assembly domain hydrophobic residues...
  15. ncbi Association of genetic variants with hemorrhagic stroke in Japanese individuals
    Tetsuro Yoshida
    Department of Cardiovascular Medicine, Inabe General Hospital, Inabe, Japan
    Int J Mol Med 25:649-56. 2010
    ....
  16. pmc SPFH1 and SPFH2 mediate the ubiquitination and degradation of inositol 1,4,5-trisphosphate receptors in muscarinic receptor-expressing HeLa cells
    Yuan Wang
    Department of Pharmacology, SUNY Upstate Medical University, Syracuse, NY 13210, USA
    Biochim Biophys Acta 1793:1710-8. 2009
    ..seeking to identify proteins that mediate IP(3) receptor ERAD we found that both SPFH1 and SPFH2 (also known as erlin 1 and erlin 2), which exist as a hetero-oligomeric complex, rapidly associate with IP(3) receptors in a manner that ..
  17. pmc When worlds collide: IP(3) receptors and the ERAD pathway
    Richard J H Wojcikiewicz
    Department of Pharmacology, SUNY Upstate Medical University, Syracuse, NY 13210, USA
    Cell Calcium 46:147-53. 2009
    ..in an unexpectedly complex manner, and that a novel complex composed of the ER membrane proteins SPFH1 and SPFH2 (erlin 1 and 2) binds to IP(3) receptors immediately after they are activated and mediates their ERAD...
  18. pmc Evolution of prokaryotic SPFH proteins
    Markus Hinderhofer
    Department of Biology, Neurobiology, University of Konstanz, Konstanz, Germany
    BMC Evol Biol 9:10. 2009
    ..To gain better insight into this very diverse protein family, we have analyzed the genomes of 497 microorganisms and investigated the pattern of occurrence as well as the genomic vicinity of the prokaryotic SPFH members...
  19. pmc Distinct regions within the erlins are required for oligomerization and association with high molecular weight complexes
    Maja B Hoegg
    Department of Biochemistry and Molecular Biology, Southern Alberta Cancer Research Institute, University of Calgary, Calgary, Alberta T2N 4N1, Canada
    J Biol Chem 284:7766-76. 2009
    ..Our data demonstrate that lower order oligomerization and incorporation into multimeric complexes are two separate biochemical properties of the erlins, because they are mediated by distinct regions...
  20. pmc Population-based genome-wide association studies reveal six loci influencing plasma levels of liver enzymes
    Xin Yuan
    Genetics Division, GlaxoSmithKline, King of Prussia, PA 19406, USA
    Am J Hum Genet 83:520-8. 2008
    ..The specific associations with ALP levels may point to genes for bone or intestinal diseases...
  21. ncbi Identification and characterization of a novel gene KE04 differentially expressed by activated human dendritic cells
    N Li
    Department of Immunology, Second Military Medical University, Shanghai, People s Republic of China
    Biochem Biophys Res Commun 279:487-93. 2000
    ..A novel full-length cDNA, KE04, was thus isolated from KLH-activated human PBMC-derived DC...

Research Grants2

  1. IP3 receptor ubiquitination and down-regulation
    Richard J H Wojcikiewicz; Fiscal Year: 2012
    ..a completely novel, and very large (>1MDa) complex composed of two uncharacterized ER membrane proteins, termed SPFH1 and 2, mediates the ERAD of activated IP3Rs and participates in the degradation of model ERAD substrates...
  2. Erlins in regulation of cholesterol-based endoplasmic reticulum functions
    LARRY R GERACE; Fiscal Year: 2012
    ..Thus, the work should shed light on a previously unrecognized mechanism involving ER nanodomains that may be crucial for regulation of cholesterol biosynthesis. ..