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Harald Jockusch

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Harald Jockusch
NationalityGerman
Known forUse of mutations and model systems to analyse the role of proteins in development, morphogenesis, and disease
Scientific career
FieldsGenetics
InstitutionsUniversity of Bielefeld

Harald Jockusch (born 1939 in Frankfurt am Main) is a German biologist and artist with the alias Hal Jos.

Scientific focus

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Scientific contributions

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RNA viruses

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During the analysis of the host defence discovery of temperature sensitive (ts) ts TMV coat proteins of tobacco mosaic virus (TMV)[1][2][3]

Relationship between the structural stability and amino acid replacements of mutant TMV coat proteins (collaboration with H.-G. Wittmann and Brigitte Wittmann-Liebold)[4]

More recent work: ts TMV coat proteins as models for misfolded (toxic) proteins in the host plant cell[5] and in transfected animal cells

Synthesis of active phage Q beta replicase in a cell-free system[6]

Developmental biology and molecular pathology

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A mouse mutant, the so-called ADR mouse, was characterized as genetic model for human myotonia type Becker.[7] As in the human Becker and Thomsen myotonias, a strongly reduced chloride conductance of the muscle fibre membrane causes hyperexcitability of the muscle fibre, leading to episodic stiffness.[8] In the ADR mouse the mutated gene was identified as coding for the then newly discovered muscular chloride channel (in collaboration with the group of Thomas Jentsch).[8][9] These results lead to the identification of the human myotonia gene mutations in which either cause recessive Becker or dominant Thomsen myotonia depending on the change in the amino acid sequence.[10]

Biomechanical analyses of the consequence of the deficiencies in the cytoskeletal proteins dystrophin (the protein deficient in Duchenne muscular dystrophy)[11] and desmin in isolated muscle fibres and muscle cell cultures[12]

Biochemical investigations into the neurodegeneration using the wobbler mouse model for ALS. The responsible gene was identified as Vps54, a gene involved in protein sorting during the retrograde vesicle transport within the cell (in collaboration with the group of Miriam Meisler, Ann Arbor)[13]

Discovery of a feedback mechanism between the extracellular protease ADAM 8 and the inflammation signalling molecule tumour necrosis factor alpha (TNFalpha) (with Jörg-Walter Bartsch)[14]

Proteomics of tissues affected by hereditary neuromuscular diseases in the mouse model; analysis of the testis in the wobbler ALS mouse model[15]

Morphogenesis of cardiac[16][17] and skeletal muscle and of the pancreas[18]

Theoretical models

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Topology of the metazoan body plan[19]

Dynamics of frequency of family names after imposing the rule "rare wins"[20]

See also

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References

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  1. ^ Jockusch, H. (1964). In vivo- und in vitro-Verhalten temperatursensitiver Mutanten des Tabakmosaikvirus. Z Vererbungsl. 1964 Dec 30;95:379-82. PMID 14315529
  2. ^ Jockusch, H. (1966). Temperatursensitive Mutanten des Tabakmosaikvirus. I. In vivo-Verhalten. Z Vererbungsl. 98, 320-343
  3. ^ Jockusch, H. (1966). Temperatursensitive Mutanten des Tabakmosaikvirus. II. In vitro-Verhalten. Z Vererbungsl. 98, 344-362
  4. ^ Jockusch H. Stability and genetic variation of a structural protein. Naturwissenschaften. 1968 Nov;55(11):514-8. No abstract available. PMID 5727497
  5. ^ Jockusch H, Wiegand C. Misfolded plant virus proteins: elicitors and targets of ubiquitylation. FEBS Lett. 2003 Jun 19;545(2-3):229-32. PMID 12804781
  6. ^ Happe M, Jockusch H. Cell-free protein synthesis resulting in active phage Qbeta replicase. Nat New Biol. 1973 Oct 3;245(144):141-3. PMID 4582894
  7. ^ Füchtbauer EM, Reininghaus, J, Jockusch, H. Developmental control of the excitability of muscle: transplantation experiments on a myotonic mouse mutant. Proc Natl Acad Sci USA. 1988 Jun; 85(11):3880-4. PMID 3375245
  8. ^ a b Mehrke G, Brinkmeier H, Jockusch H. The myotonic mouse mutant ADR: electrophysiology of the muscle fiber. Muscle Nerve. 1988 May; 11(5):440-6. PMID 2453798
  9. ^ Steinmeyer K, Klocke R, Ortland C, Gronemeier M, Jockusch H, Gründer S, Jentsch TJ. Inactivation of muscle chloride channel by transposon insertion in myotonic mice. Nature. 1991 Nov 28;354(6351):304-8. PMID 1659665)
  10. ^ Koch MC, Steinmeyer K, Lorenz C, Ricker K, Wolf F, Otto M, Zoll B, Lehmann-Horn F, Grzeschik KH, Jentsch TJ. The skeletal muscle chloride channel in dominant and recessive human myotonia. Science. 1992 Aug 7;257(5071):797-800. PMID 1379744
  11. ^ Menke A, Jockusch H. Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse. Nature. 1991 Jan 3;349(6304):69-71.PMID 1985268)
  12. ^ Wieneke S, Stehle R, Li Z, Jockusch H. Generation of tension by skinned fibers and intact skeletal muscles from desmin-deficient mice. Biochem Biophys Res Commun. 2000 Nov 19;278(2):419-25.PMID 11097852 [PubMed – indexed for MEDLINE
  13. ^ Schmitt-John T, Drepper C, Mussmann A, Hahn P, Kuhlmann M, Thiel C, Hafner M, Lengeling A, Heimann P, Jones JM, Meisler MH, Jockusch H. Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse. Nat Genet. 2005 Nov;37(11):1213-5. Epub 2005 Oct 23. PMID 16244655
  14. ^ Bartsch JW, Wildeboer D, Koller G, Naus S, Rittger A, Moss ML, Minai Y, Jockusch H. Tumor necrosis factor-alpha (TNF-alpha) regulates shedding of TNF-alpha receptor 1 by the metalloprotease-disintegrin ADAM8: evidence for a protease-regulated feedback loop in neuroprotection. J Neurosci. 2010 Sep 8;30(36):12210-8. doi: 10.1523/JNEUROSCI.1520-10.2010.PMID 20826683
  15. ^ Jockusch H, Holland A, Staunton L, Schmitt-John T, Heimann P, Dowling P, Ohlendieck K. Pathoproteomics of testicular tissue deficient in the GARP component VPS54: The wobbler mouse model of globozoospermia. Proteomics. 2013 Sep 30. doi: 10.1002/pmic.201300189. [Epub ahead of print] PMID 24115398
  16. ^ Jockusch H, Füchtbauer EM, Füchtbauer A, Léger JJ, Léger J, Maldonado CA, Forssmann WG. Long-term expression of isomyosins and myoendocrine functions in ectopic grafts of atrial tissue. Proc Natl Acad Sci U S A. 1986 Oct;83(19):7325-9. PMID 3463971
  17. ^ Eberhard D, Jockusch H. Patterns of myocardial histogenesis as revealed by mouse chimeras. Dev Biol. 2005 Feb15;278(2):336-46. PMID 15680354
  18. ^ Eberhard D, Jockusch H. Clonal and territorial development of the pancreas as revealed by eGFP-labelled mouse chimeras. Cell Tissue Res. 2010 Oct;342(1):31-8. doi: 10.1007/s00441-010-1028-y. Epub 2010 Aug 29. PMID 20803297
  19. ^ Jockusch H, Dress A. From sphere to torus: a topological view of the metazoan body plan. Bull Math Biol. 2003 Jan;65(1):57-65.PMID 12597116
  20. ^ Jockusch H, Fuhrmann A (2010). „Selten gewinnt" – Rare wins. Changes in the frequencies of family names as a consequence of rational choice. Beiträge zur Namenforschung 45(2): 127–142)
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