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PAM18 Pam18p

Pam18, Tim14, DNAJC19
The protein encoded by this gene is thought to be part of a complex involved in the ATP-dependent transport of transit peptide-containing proteins from the inner cell membrane to the mitochondrial matrix. Defects in this gene are a cause of 3-methylglutaconic aciduria type 5 (MGA5), also known as dilated cardiomyopathy with ataxia (DCMA). Alternative splicing of this gene results in multiple transcript variants. Related pseudogenes have been identified on chromosomes 1, 2, 6, 10, 14 and 19. [provided by RefSeq, Jan 2012] (from NCBI)
Top mentioned proteins: ATPase, HSP70, ACID, STEP, V1a
Papers on Pam18
DNAJC19, a mitochondrial cochaperone associated with cardiomyopathy, forms a complex with prohibitins to regulate cardiolipin remodeling.
Langer et al., Köln, Germany. In Cell Metab, 2014
Here, we have defined the interactome of PHB2 in mitochondria and identified DNAJC19, mutated in dilated cardiomyopathy with ataxia, as binding partner of PHB complexes.
Remodelling of the active presequence translocase drives motor-dependent mitochondrial protein translocation.
Rehling et al., Göttingen, Germany. In Nat Commun, 2013
Here we establish an assay to monitor protein dynamics in the precursor-occupied presequence translocase and find that regulatory subunits of the import motor, such as the ATPase-stimulating J-protein Pam18, are recruited into the translocation intermediate.
Leucine Loading Test is Only Discriminative for 3-Methylglutaconic Aciduria Due to AUH Defect.
Morava et al., Nijmegen, Netherlands. In Jimd Rep, 2013
For all other subtypes, also denoted "Secondary 3-methylglutaconic acidurias" (TAZ defect or Barth syndrome, SERAC1 defect or MEGDEL syndrome, OPA3 defect or Costeff syndrome, DNAJC19 defect or DCMA syndrome, TMEM70 defect, "not otherwise specified (NOS) 3-MGA-uria"), the origin of 3-methylglutaconic aciduria remains enigmatic but is hypothesized to be independent from leucine catabolism.
3-Methylglutaconic aciduria--lessons from 50 genes and 977 patients.
Wevers et al., Nijmegen, Netherlands. In J Inherit Metab Dis, 2013
Besides, it was a consistent feature of patients with mutations in TAZ, SERAC1, OPA3, DNAJC19 and TMEM70 accounting for mitochondrial membrane related pathology.
Inborn errors of metabolism with 3-methylglutaconic aciduria as discriminative feature: proper classification and nomenclature.
Wevers et al., Nijmegen, Netherlands. In J Inherit Metab Dis, 2013
The latter should be further classified and named by their defective protein or the historical name as follows: i) defective phospholipid remodelling (TAZ defect or Barth syndrome, SERAC1 defect or MEGDEL syndrome) and ii) mitochondrial membrane associated disorders (OPA3 defect or Costeff syndrome, DNAJC19 defect or DCMA syndrome, TMEM70 defect).
Exome sequencing identifies a new mutation in SERAC1 in a patient with 3-methylglutaconic aciduria.
Ribes et al., Barcelona, Spain. In Mol Genet Metab, 2013
Causative mutations in TAZ, OPA3, DNAJC19, ATP12, ATP5E, and TMEM70 have been identified.
Methylation-controlled J-protein MCJ acts in the import of proteins into human mitochondria.
Hell et al., München, Germany. In Hum Mol Genet, 2013
Interestingly, MCJ is able to take over the function of Tim14, the essential J co-chaperone of the mitochondrial protein import motor in yeast.
Comparative analysis of putative orthologues of mitochondrial import motor subunit: Pam18 and Pam16 in plants.
Cheng et al., China. In Plos One, 2012
Pam18/Tim14 and Pam16/Tim16, highly conserved proteins among eukaryotes, are two essential subunits of protein import motors localized in the inner mitochondrial membrane.
New mutation of mitochondrial DNAJC19 causing dilated and noncompaction cardiomyopathy, anemia, ataxia, and male genital anomalies.
Tyni et al., Helsinki, Finland. In Pediatr Res, 2012
BACKGROUND: We report a new mutation in the human DNAJC19 gene that causes early onset dilated cardiomyopathy syndrome (DCMA).
Genetic analysis of complex interactions among components of the mitochondrial import motor and translocon in Saccharomyces cerevisiae.
Craig et al., Madison, United States. In Genetics, 2012
Pam18, the J-protein co-chaperone, and Pam16, a structurally related protein with which Pam18 forms a heterodimer, are also critical components of the motor.
Brain region-specific altered expression and association of mitochondria-related genes in autism.
Mori et al., Hamamatsu, Japan. In Mol Autism, 2011
The expression of DNAJC19, DNM1L, LRPPRC, SLC25A12, SLC25A14, SLC25A24 and TOMM20 were reduced in at least two of the brain regions of autism patients.
Reevaluation of the role of the Pam18:Pam16 interaction in translocation of proteins by the mitochondrial Hsp70-based import motor.
Craig et al., Madison, United States. In Mol Biol Cell, 2011
Analysis of a previously uncharacterized region of Pam16 revealed its requirement for formation of an active Pam18:Pam16 complex able to stimulate Hsp70's ATPase activity.
Ancient gene duplication provided a key molecular step for anaerobic growth of Baker's yeast.
Craig et al., Madison, United States. In Mol Biol Evol, 2011
In Saccharomyces cerevisiae, two paralogous J-proteins, Pam18 and Mdj2, can form the import motor.
The core components of organelle biogenesis and membrane transport in the hydrogenosomes of Trichomonas vaginalis.
Tachezy et al., Praha, Czech Republic. In Plos One, 2010
The inner membrane proteins TvTim17/22/23-1 and Pam18 were shown to possess conserved information for targeting to mitochondrial inner membranes, but too divergent in sequence to support the growth of yeast strains lacking Tim17, Tim22, Tim23 or Pam18.
Interaction of the J-protein heterodimer Pam18/Pam16 of the mitochondrial import motor with the translocon of the inner membrane.
Craig et al., Madison, United States. In Mol Biol Cell, 2008
These data suggest a model in which Tim44 serves as a scaffold for precise positioning of mtHsp70 and its cochaperone Pam18 at the translocon.
The interplay between components of the mitochondrial protein translocation motor studied using purified components.
Azem et al., Tel Aviv-Yafo, Israel. In J Biol Chem, 2007
binding of Tim44 to mHsp70 prevents the formation of a complex between the latter and Tim14/Pam18-Tim16/Pam16.
Association of the Tim14.Tim16 subcomplex with the TIM23 translocase is crucial for function of the mitochondrial protein import motor.
Hell et al., München, Germany. In J Biol Chem, 2007
import into the matrix space of mitochondria requires association of the co-chaperones Tim16 and Tim14 with the TIM23 preprotein translocase
Cardiac features of a novel autosomal recessive dilated cardiomyopathic syndrome due to defective importation of mitochondrial protein.
Bernier et al., Calgary, Canada. In Cardiol Young, 2007
Dilated cardiomyopathy caused by homozygous mutations in a novel gene, DNAJC19, presumed to play a role in importation of mitochondrial proteins.
Mitochondrial presequence translocase: switching between TOM tethering and motor recruitment involves Tim21 and Tim17.
Rehling et al., Freiburg, Germany. In Cell, 2005
Tim17 is crucial for the switch by performing two separable functions: promotion of inner membrane insertion and binding of Pam18 to form the functional TIM-PAM complex.
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