Autocatalytic processing of m-AAA protease subunits in mitochondria.

Abstract:

:m-AAA proteases are ATP-dependent proteolytic machines in the inner membrane of mitochondria which are crucial for the maintenance of mitochondrial activities. Conserved nuclear-encoded subunits, termed paraplegin, Afg3l1, and Afg3l2, form various isoenzymes differing in their subunit composition in mammalian mitochondria. Mutations in different m-AAA protease subunits are associated with distinct neuronal disorders in human. However, the biogenesis of m-AAA protease complexes or of individual subunits is only poorly understood. Here, we have examined the processing of nuclear-encoded m-AAA protease subunits upon import into mitochondria and demonstrate autocatalytic processing of Afg3l1 and Afg3l2. The mitochondrial processing peptidase MPP generates an intermediate form of Afg3l2 that is matured autocatalytically. Afg3l1 or Afg3l2 are also required for maturation of newly imported paraplegin subunits after their cleavage by MPP. Our results establish that mammalian m-AAA proteases can act as processing enzymes in vivo and reveal overlapping activities of Afg3l1 and Afg3l2. These findings might be of relevance for the pathogenesis of neurodegenerative disorders associated with mutations in different m-AAA protease subunits.

journal_name

Mol Biol Cell

authors

Koppen M,Bonn F,Ehses S,Langer T

doi

10.1091/mbc.e09-03-0218

subject

Has Abstract

pub_date

2009-10-01 00:00:00

pages

4216-24

issue

19

eissn

1059-1524

issn

1939-4586

pii

E09-03-0218

journal_volume

20

pub_type

杂志文章
  • ATP-dependent membrane assembly of F-actin facilitates membrane fusion.

    abstract::We recently established an in vitro assay that monitors the fusion between latex-bead phagosomes and endocytic organelles in the presence of J774 macrophage cytosol (). Here, we show that different reagents affecting the actin cytoskeleton can either inhibit or stimulate this fusion process. Because the membranes of p...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.12.1.155

    authors: Jahraus A,Egeberg M,Hinner B,Habermann A,Sackman E,Pralle A,Faulstich H,Rybin V,Defacque H,Griffiths G

    更新日期:2001-01-01 00:00:00

  • A double-strand break repair component is essential for S phase completion in fission yeast cell cycling.

    abstract::Fission yeast rad22(+), a homologue of budding yeast RAD52, encodes a double-strand break repair component, which is dispensable for proliferation. We, however, have recently obtained a cell division cycle mutant with a temperature-sensitive allele of rad22(+), designated rad22-H6, which resulted from a point mutation...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.10.10.3331

    authors: Suto K,Nagata A,Murakami H,Okayama H

    更新日期:1999-10-01 00:00:00

  • Essential role of cyclin-G-associated kinase (Auxilin-2) in developing and mature mice.

    abstract::Hsc70 with its cochaperone, either auxilin or GAK, not only uncoats clathrin-coated vesicles but also acts as a chaperone during clathrin-mediated endocytosis. However, because synaptojanin is also involved in uncoating, it is not clear whether GAK is an essential gene. To answer this question, GAK conditional knockou...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.e07-11-1115

    authors: Lee DW,Zhao X,Yim YI,Eisenberg E,Greene LE

    更新日期:2008-07-01 00:00:00

  • Mice lacking mannose 6-phosphate uncovering enzyme activity have a milder phenotype than mice deficient for N-acetylglucosamine-1-phosphotransferase activity.

    abstract::The mannose 6-phosphate (Man-6-P) lysosomal targeting signal on acid hydrolases is synthesized by the sequential action of uridine 5'-diphosphate-N-acetylglucosamine: lysosomal enzyme N-acetylglucosamine-1-phosphotransferase (GlcNAc-1-phosphotransferase) and GlcNAc-1-phosphodiester alpha-N-acetylglucosaminidase ("unco...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.e09-05-0398

    authors: Boonen M,Vogel P,Platt KA,Dahms N,Kornfeld S

    更新日期:2009-10-01 00:00:00

  • Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway.

    abstract::Small heat shock proteins (sHsps) bind destabilized proteins during cell stress and disease, but their physiological functions are less clear. We evaluated the impact of Hsp27, an sHsp expressed in airway epithelial cells, on the common protein misfolding mutant that is responsible for most cystic fibrosis. F508del cy...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E12-09-0678

    authors: Ahner A,Gong X,Schmidt BZ,Peters KW,Rabeh WM,Thibodeau PH,Lukacs GL,Frizzell RA

    更新日期:2013-01-01 00:00:00

  • RhoA is dispensable for skin development, but crucial for contraction and directed migration of keratinocytes.

    abstract::RhoA is a small guanosine-5'-triphosphatase (GTPase) suggested to be essential for cytokinesis, stress fiber formation, and epithelial cell-cell contacts. In skin, loss of RhoA was suggested to underlie pemphigus skin blistering. To analyze RhoA function in vivo, we generated mice with a keratinocyte-restricted deleti...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E09-10-0859

    authors: Jackson B,Peyrollier K,Pedersen E,Basse A,Karlsson R,Wang Z,Lefever T,Ochsenbein AM,Schmidt G,Aktories K,Stanley A,Quondamatteo F,Ladwein M,Rottner K,van Hengel J,Brakebusch C

    更新日期:2011-03-01 00:00:00

  • The cardiolipin transacylase, tafazzin, associates with two distinct respiratory components providing insight into Barth syndrome.

    abstract::Mutations in the mitochondrial cardiolipin (CL) transacylase, tafazzin (Taz1p), result in the X-linked cardioskeletal myopathy, Barth syndrome (BTHS). The mitochondria of BTHS patients exhibit variable respiratory defects and abnormal cristae ultrastructure. The biochemical basis for these observations is unknown. In ...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.e08-09-0896

    authors: Claypool SM,Boontheung P,McCaffery JM,Loo JA,Koehler CM

    更新日期:2008-12-01 00:00:00

  • Repositioning of muscle-specific genes relative to the periphery of SC-35 domains during skeletal myogenesis.

    abstract::Previous studies have shown that in a given cell type, certain active genes associate with SC-35 domains, nuclear regions rich in RNA metabolic factors and excluded from heterochromatin. This organization is not seen for all active genes; therefore, it is important to determine whether and when this locus-specific org...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.e03-06-0388

    authors: Moen PT Jr,Johnson CV,Byron M,Shopland LS,de la Serna IL,Imbalzano AN,Lawrence JB

    更新日期:2004-01-01 00:00:00

  • Direct binding of the ligand PSG17 to CD9 requires a CD9 site essential for sperm-egg fusion.

    abstract::The function currently attributed to tetraspanins is to organize molecular complexes in the plasma membrane by using multiple cis-interactions. Additionally, the tetraspanin CD9 may be a receptor that binds the soluble ligand PSG17, a member of the immunoglobulin superfamily (IgSF)/CEA subfamily. However, previous dat...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.e03-04-0244

    authors: Ellerman DA,Ha C,Primakoff P,Myles DG,Dveksler GS

    更新日期:2003-12-01 00:00:00

  • Infrequent translation of a nonsense codon is sufficient to decrease mRNA level.

    abstract::In many organisms nonsense mutations decrease the level of mRNA. In the case of mammalian cells, it is still controversial whether translation is required for this nonsense-mediated RNA decrease (NMD). Although previous analyzes have shown that conditions that impede translation termination at nonsense codons also pre...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.10.3.515

    authors: Buzina A,Shulman MJ

    更新日期:1999-03-01 00:00:00

  • The DNA helicase activity of BLM is necessary for the correction of the genomic instability of bloom syndrome cells.

    abstract::Bloom syndrome (BS) is a rare autosomal recessive disorder characterized by growth deficiency, immunodeficiency, genomic instability, and the early development of cancers of many types. BLM, the protein encoded by BLM, the gene mutated in BS, is localized in nuclear foci and absent from BS cells. BLM encodes a DNA hel...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.10.3.665

    authors: Neff NF,Ellis NA,Ye TZ,Noonan J,Huang K,Sanz M,Proytcheva M

    更新日期:1999-03-01 00:00:00

  • Laminin 511 partners with laminin 332 to mediate directional migration of Madin-Darby canine kidney epithelial cells.

    abstract::Sustained directional migration of epithelial cells is essential for regeneration of injured epithelia. Front-rear polarity of migrating cells is determined by local activation of a signaling network involving Cdc42 and other factors in response to spatial cues from the environment, the nature of which are obscure. We...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E11-08-0718

    authors: Greciano PG,Moyano JV,Buschmann MM,Tang J,Lu Y,Rudnicki J,Manninen A,Matlin KS

    更新日期:2012-01-01 00:00:00

  • Wounded cells drive rapid epidermal repair in the early Drosophila embryo.

    abstract::Epithelial tissues are protective barriers that display a remarkable ability to repair wounds. Wound repair is often associated with an accumulation of actin and nonmuscle myosin II around the wound, forming a purse string. The role of actomyosin networks in generating mechanical force during wound repair is not well ...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E13-05-0228

    authors: Fernandez-Gonzalez R,Zallen JA

    更新日期:2013-10-01 00:00:00

  • A novel transcriptional enhancer is involved in the prolactin- and extracellular matrix-dependent regulation of beta-casein gene expression.

    abstract::Lactogenic hormones and extracellular matrix (ECM) act synergistically to regulate beta-casein expression in culture. We have developed a functional subpopulation of the mouse mammary epithelial cell strain COMMA-1D (designated CID 9), which expresses high level of beta-casein, forms alveolar-like structures when plat...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.3.6.699

    authors: Schmidhauser C,Casperson GF,Myers CA,Sanzo KT,Bolten S,Bissell MJ

    更新日期:1992-06-01 00:00:00

  • Structure of Shroom domain 2 reveals a three-segmented coiled-coil required for dimerization, Rock binding, and apical constriction.

    abstract::Shroom (Shrm) proteins are essential regulators of cell shape and tissue morpho-logy during animal development that function by interacting directly with the coiled-coil region of Rho kinase (Rock). The Shrm-Rock interaction is sufficient to direct Rock subcellular localization and the subsequent assembly of contracti...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E11-11-0937

    authors: Mohan S,Rizaldy R,Das D,Bauer RJ,Heroux A,Trakselis MA,Hildebrand JD,VanDemark AP

    更新日期:2012-06-01 00:00:00

  • Multilayered regulation of TORC1-body formation in budding yeast.

    abstract::The target of rapamycin kinase complex 1 (TORC1) regulates cell growth and metabolism in eukaryotes. In Saccharomyces cerevisiae, TORC1 activity is known to be controlled by the conserved GTPases, Gtr1/2, and movement into and out of an inactive agglomerate/body. However, it is unclear whether/how these regulatory ste...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E18-05-0297

    authors: Sullivan A,Wallace RL,Wellington R,Luo X,Capaldi AP

    更新日期:2019-02-01 00:00:00

  • Actin-binding protein G (AbpG) participates in modulating the actin cytoskeleton and cell migration in Dictyostelium discoideum.

    abstract::Cell migration is involved in various physiological and pathogenic events, and the complex underlying molecular mechanisms have not been fully elucidated. The simple eukaryote Dictyostelium discoideum displays chemotactic locomotion in stages of its life cycle. By characterizing a Dictyostelium mutant defective in che...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E14-05-0972

    authors: Lin WC,Wang LC,Pang TL,Chen MY

    更新日期:2015-03-15 00:00:00

  • Regulation of macropinocytosis by p21-activated kinase-1.

    abstract::The process of macropinocytosis is an essential aspect of normal cell function, contributing to both growth and motile processes of cells. p21-activated kinases (PAKs) are targets for activated Rac and Cdc42 guanosine 5'-triphosphatases and have been shown to regulate the actin-myosin cytoskeleton. In fibroblasts PAK1...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.11.10.3341

    authors: Dharmawardhane S,Schürmann A,Sells MA,Chernoff J,Schmid SL,Bokoch GM

    更新日期:2000-10-01 00:00:00

  • Temporal control of nuclear envelope assembly by phosphorylation of lamin B receptor.

    abstract::The nuclear envelope of metazoans disassembles during mitosis and reforms in late anaphase after sister chromatids have well separated. The coordination of these mitotic events is important for genome stability, yet the temporal control of nuclear envelope reassembly is unknown. Although the steps of nuclear formation...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E11-03-0199

    authors: Tseng LC,Chen RH

    更新日期:2011-09-01 00:00:00

  • Casein kinase II and calcineurin modulate TRPP function and ciliary localization.

    abstract::Cilia serve as sensory devices in a diversity of organisms and their defects contribute to many human diseases. In primary cilia of kidney cells, the transient receptor potential polycystin (TRPP) channels polycystin-1 (PC-1) and polycystin-2 (PC-2) act as a mechanosensitive channel, with defects resulting in autosoma...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.e05-10-0935

    authors: Hu J,Bae YK,Knobel KM,Barr MM

    更新日期:2006-05-01 00:00:00

  • Intrinsically active variants of Erk oncogenically transform cells and disclose unexpected autophosphorylation capability that is independent of TEY phosphorylation.

    abstract::The receptor-tyrosine kinase (RTK)/Ras/Raf pathway is an essential cascade for mediating growth factor signaling. It is abnormally overactive in almost all human cancers. The downstream targets of the pathway are members of the extracellular regulated kinases (Erk1/2) family, suggesting that this family is a mediator ...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E15-07-0521

    authors: Smorodinsky-Atias K,Goshen-Lago T,Goldberg-Carp A,Melamed D,Shir A,Mooshayef N,Beenstock J,Karamansha Y,Darlyuk-Saadon I,Livnah O,Ahn NG,Admon A,Engelberg D

    更新日期:2016-03-15 00:00:00

  • A prefoldin-associated WD-repeat protein (WDR92) is required for the correct architectural assembly of motile cilia.

    abstract::WDR92 is a highly conserved WD-repeat protein that has been proposed to be involved in apoptosis and also to be part of a prefoldin-like cochaperone complex. We found that WDR92 has a phylogenetic signature that is generally compatible with it playing a role in the assembly or function of specifically motile cilia. To...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E16-01-0040

    authors: Patel-King RS,King SM

    更新日期:2016-04-15 00:00:00

  • Estrogen regulates histone deacetylases to prevent cardiac hypertrophy.

    abstract::The development and progression of cardiac hypertrophy often leads to heart failure and death, and important modulators of hypertrophy include the histone deacetylase proteins (HDACs). Estrogen inhibits cardiac hypertrophy and progression in animal models and humans. We therefore investigated the influence of 17-β-est...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E13-08-0444

    authors: Pedram A,Razandi M,Narayanan R,Dalton JT,McKinsey TA,Levin ER

    更新日期:2013-12-01 00:00:00

  • The same receptor, G protein, and mitogen-activated protein kinase pathway activate different downstream regulators in the alternative white and opaque pheromone responses of Candida albicans.

    abstract::Candida albicans must undergo a switch from white to opaque to mate. Opaque cells then release mating type-specific pheromones that induce mating responses in opaque cells. Uniquely in C. albicans, the same pheromones induce mating-incompetent white cells to become cohesive, form an adhesive basal layer of cells on a ...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.e07-07-0688

    authors: Yi S,Sahni N,Daniels KJ,Pujol C,Srikantha T,Soll DR

    更新日期:2008-03-01 00:00:00

  • Down-regulation of cell surface receptors is modulated by polar residues within the transmembrane domain.

    abstract::How recycling receptors are segregated from down-regulated receptors in the endosome is unknown. In previous studies, we demonstrated that substitutions in the transferrin receptor (TR) transmembrane domain (TM) convert the protein from an efficiently recycling receptor to one that is rapidly down regulated. In this s...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.11.8.2643

    authors: Zaliauskiene L,Kang S,Brouillette CG,Lebowitz J,Arani RB,Collawn JF

    更新日期:2000-08-01 00:00:00

  • Protein interactions with the glucose transporter binding protein GLUT1CBP that provide a link between GLUT1 and the cytoskeleton.

    abstract::Subcellular targeting and the activity of facilitative glucose transporters are likely to be regulated by interactions with cellular proteins. This report describes the identification and characterization of a protein, GLUT1 C-terminal binding protein (GLUT1CBP), that binds via a PDZ domain to the C terminus of GLUT1....

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.10.4.819

    authors: Bunn RC,Jensen MA,Reed BC

    更新日期:1999-04-01 00:00:00

  • In vivo evidence for cooperation of Mia40 and Erv1 in the oxidation of mitochondrial proteins.

    abstract::The intermembrane space of mitochondria accommodates the essential mitochondrial intermembrane space assembly (MIA) machinery that catalyzes oxidative folding of proteins. The disulfide bond formation pathway is based on a relay of reactions involving disulfide transfer from the sulfhydryl oxidase Erv1 to Mia40 and fr...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E12-05-0358

    authors: Böttinger L,Gornicka A,Czerwik T,Bragoszewski P,Loniewska-Lwowska A,Schulze-Specking A,Truscott KN,Guiard B,Milenkovic D,Chacinska A

    更新日期:2012-10-01 00:00:00

  • Cross-talk between androgen receptor/filamin A and TrkA regulates neurite outgrowth in PC12 cells.

    abstract::Steroids and growth factors control neuronal development through their receptors under physiological and pathological conditions. We show that PC12 cells harbor endogenous androgen receptor (AR), whose inhibition or silencing strongly interferes with neuritogenesis stimulated by the nonaromatizable synthetic androgen ...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E14-09-1352

    authors: Di Donato M,Bilancio A,D'Amato L,Claudiani P,Oliviero MA,Barone MV,Auricchio A,Appella E,Migliaccio A,Auricchio F,Castoria G

    更新日期:2015-08-01 00:00:00

  • Distinct roles for the AAA ATPases NSF and p97 in the secretory pathway.

    abstract::NSF and p97 are related AAA proteins implicated in membrane trafficking and organelle biogenesis. p97 is also involved in pathways that lead to ubiquitin-dependent proteolysis, including ER-associated degradation (ERAD). In this study, we have used dominant interfering ATP-hydrolysis deficient mutants (NSF(E329Q) and ...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.e03-02-0097

    authors: Dalal S,Rosser MF,Cyr DM,Hanson PI

    更新日期:2004-02-01 00:00:00

  • Reticulon 4a promotes exocytosis in mammalian cells.

    abstract::Endoplasmic reticulum (ER) tubules and sheets conventionally correspond to smooth and rough ER, respectively. The ratio of ER tubules-to-sheets varies in different cell types and changes in response to cellular conditions, potentially impacting the functional output of the ER. To directly test whether ER morphology im...

    journal_title:Molecular biology of the cell

    pub_type: 杂志文章

    doi:10.1091/mbc.E19-03-0159

    authors: Mukherjee RN,Levy DL

    更新日期:2019-08-15 00:00:00