Proteolytic control of mitochondrial protein biogenesis:
Saved in:
| Main Author: | |
|---|---|
| Format: | Thesis Book |
| Language: | English |
| Published: |
Freiburg im Breisgau
July 2020
|
| Subjects: | |
| Online Access: | Inhaltsverzeichnis Inhaltsverzeichnis |
| Item Description: | Enthält Zeitschriftenaufsätze |
| Physical Description: | 176 Seiten Illustrationen, Diagramme 30 cm |
Staff View
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| 245 | 1 | 0 | |a Proteolytic control of mitochondrial protein biogenesis |c presented by Cansu Kücükköse |
| 264 | 1 | |a Freiburg im Breisgau |c July 2020 | |
| 300 | |a 176 Seiten |b Illustrationen, Diagramme |c 30 cm | ||
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Record in the Search Index
| _version_ | 1804182743759716352 |
|---|---|
| adam_text | TABLE
OF
CONTENTS
LIST
OF
ABBREVIATIONS
............................................................................................
ILL
1.
SUMMARY.
.................................................................................
1
2.
INTRODUCTION
........................................................................................................
2
2.1.
MITOCHONDRIA:
FROM
ORIGIN
TO
FUNCTION
........................................................................
2
2.2.
MITOCHONDRIAL
PROTEIN
BIOGENESIS
-
A
GENERAL
OVERVIEW
...........................................
3
2.3.
MITOCHONDRIAL
PRESEQUENCE
IMPORT
PATHWAY
.............................................................
5
2.4.
MITOCHONDRIAL
PRESEQUENCE
PROCESSING
MACHINERY
.................................................
8
2.5.
MITOCHONDRIAL
QUALITY
CONTROL:
PRESEQUENCE
DEGRADATION
AND
PROTEIN
TURNOVER
..10
2.6.
MITOCHONDRIAL
UNFOLDED
PROTEIN
RESPONSE
(MTUPR)
...............................................
13
2.7.
ROLE
OF
MITOCHONDRIAL
PROTEASES
IN
NEURODEGENERATIVE
DISEASES
.........................
14
3.
AIM
OF
THIS
STUDY
..............................................................................................
16
4.
RESULTS
AND
DISCUSSION
.................................................................................
17
4.1
IDENTIFICATION
AND
CHARACTERIZATION
OF
NOVEL
PRESEQUENCE
PROTEASES
....................
17
4.1.1
CHARACTERIZATION
OF
THE
HUMAN
INTERMEDIATE
CLEAVING
PEPTIDASE
ICP55
...........
17
4.1.2
CHARACTERIZATION
OF
THE
PRESEQUENCE
PEPTIDASE
(PREP)
IN
HUMAN
CELLS
...........
22
4.1.3
IDENTIFICATION
OF
THE
NOVEL
MITOCHONDRIAL
MATRIX
PEPTIDASE
STE23
.....................
24
4.1.4
CHARACTERIZATION
OF
THE
PEPTIDE
DEGRADING
ENZYME
PRD1
...................................
26
4.2
FUNCTIONAL
COUPLING
OF
PRECURSOR
PROCESSING
AND
PRESEQUENCE
DEGRADATION
.....
29
4.3
MUTATIONS
IN
PRESEQUENCE
PROTEASES
CAUSE
NEURODEGENERATION
..........................
32
4.4
IMPAIRED
MITOCHONDRIAL
PROTEOSTASIS
TRIGGERS
A
PROTECTIVE
STRESS
RESPONSE
........
36
5.
MATERIALS
AND
METHODS
...................................................................................
40
6.
REFERENCES
.......................................................................................................
51
7.
PUBLICATIONS
.....................................................................................................62
7.1
FUNCTIONAL
COUPLING
OF
PRESEQUENCE
PROCESSING
AND
DEGRADATION
IN
HUMAN
MITOCHONDRIA
.....................................................................................................................
64
7.2
THE
NOVEL
MITOCHONDRIAL
MATRIX
PROTEASE
STE23
IS
REQUIRED
FOR
EFFICIENT
PRESEQUENCE
DEGRADATION
AND
PROCESSING
....................................................................
78
7.3
LANDSCAPE
OF
SUBMITOCHONDRIAL
PROTEIN
DISTRIBUTION
...............................................
89
7.4
MUTATIONS
IN
PMPCB
ENCODING
THE
CATALYTIC
SUBUNIT
OF
THE
MITOCHONDRIAL
PRESEQUENCE
PROTEASE
CAUSE
NEURODEGENERATION
IN
EARLY
CHILDHOOD
........................
113
7.5
AN
EARLY
MTUPR:
REDISTRIBUTION
OF
THE
NUCLEAR
TRANSCRIPTION
FACTOR
ROX1
TO
MITOCHONDRIA
PROTECTS
AGAINST
INTRAMITOCHONDRIAL
PROTEOTOXIC
AGGREGATES
................
148
|
| adam_txt |
TABLE
OF
CONTENTS
LIST
OF
ABBREVIATIONS
.
ILL
1.
SUMMARY.
.
1
2.
INTRODUCTION
.
2
2.1.
MITOCHONDRIA:
FROM
ORIGIN
TO
FUNCTION
.
2
2.2.
MITOCHONDRIAL
PROTEIN
BIOGENESIS
-
A
GENERAL
OVERVIEW
.
3
2.3.
MITOCHONDRIAL
PRESEQUENCE
IMPORT
PATHWAY
.
5
2.4.
MITOCHONDRIAL
PRESEQUENCE
PROCESSING
MACHINERY
.
8
2.5.
MITOCHONDRIAL
QUALITY
CONTROL:
PRESEQUENCE
DEGRADATION
AND
PROTEIN
TURNOVER
.10
2.6.
MITOCHONDRIAL
UNFOLDED
PROTEIN
RESPONSE
(MTUPR)
.
13
2.7.
ROLE
OF
MITOCHONDRIAL
PROTEASES
IN
NEURODEGENERATIVE
DISEASES
.
14
3.
AIM
OF
THIS
STUDY
.
16
4.
RESULTS
AND
DISCUSSION
.
17
4.1
IDENTIFICATION
AND
CHARACTERIZATION
OF
NOVEL
PRESEQUENCE
PROTEASES
.
17
4.1.1
CHARACTERIZATION
OF
THE
HUMAN
INTERMEDIATE
CLEAVING
PEPTIDASE
ICP55
.
17
4.1.2
CHARACTERIZATION
OF
THE
PRESEQUENCE
PEPTIDASE
(PREP)
IN
HUMAN
CELLS
.
22
4.1.3
IDENTIFICATION
OF
THE
NOVEL
MITOCHONDRIAL
MATRIX
PEPTIDASE
STE23
.
24
4.1.4
CHARACTERIZATION
OF
THE
PEPTIDE
DEGRADING
ENZYME
PRD1
.
26
4.2
FUNCTIONAL
COUPLING
OF
PRECURSOR
PROCESSING
AND
PRESEQUENCE
DEGRADATION
.
29
4.3
MUTATIONS
IN
PRESEQUENCE
PROTEASES
CAUSE
NEURODEGENERATION
.
32
4.4
IMPAIRED
MITOCHONDRIAL
PROTEOSTASIS
TRIGGERS
A
PROTECTIVE
STRESS
RESPONSE
.
36
5.
MATERIALS
AND
METHODS
.
40
6.
REFERENCES
.
51
7.
PUBLICATIONS
.62
7.1
FUNCTIONAL
COUPLING
OF
PRESEQUENCE
PROCESSING
AND
DEGRADATION
IN
HUMAN
MITOCHONDRIA
.
64
7.2
THE
NOVEL
MITOCHONDRIAL
MATRIX
PROTEASE
STE23
IS
REQUIRED
FOR
EFFICIENT
PRESEQUENCE
DEGRADATION
AND
PROCESSING
.
78
7.3
LANDSCAPE
OF
SUBMITOCHONDRIAL
PROTEIN
DISTRIBUTION
.
89
7.4
MUTATIONS
IN
PMPCB
ENCODING
THE
CATALYTIC
SUBUNIT
OF
THE
MITOCHONDRIAL
PRESEQUENCE
PROTEASE
CAUSE
NEURODEGENERATION
IN
EARLY
CHILDHOOD
.
113
7.5
AN
EARLY
MTUPR:
REDISTRIBUTION
OF
THE
NUCLEAR
TRANSCRIPTION
FACTOR
ROX1
TO
MITOCHONDRIA
PROTECTS
AGAINST
INTRAMITOCHONDRIAL
PROTEOTOXIC
AGGREGATES
.
148 |
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| author | Kücükköse, Cansu |
| author_GND | (DE-588)1225001056 |
| author_facet | Kücükköse, Cansu |
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| author_sort | Kücükköse, Cansu |
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| building | Verbundindex |
| bvnumber | BV047448101 |
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| discipline | Biologie |
| discipline_str_mv | Biologie |
| format | Thesis Book |
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| index_date | 2024-07-03T18:02:54Z |
| indexdate | 2024-07-10T09:12:24Z |
| institution | BVB |
| language | English |
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| owner_facet | DE-355 DE-BY-UBR |
| physical | 176 Seiten Illustrationen, Diagramme 30 cm |
| publishDate | 2020 |
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| publishDateSort | 2020 |
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| spelling | Kücükköse, Cansu Verfasser (DE-588)1225001056 aut Proteolytic control of mitochondrial protein biogenesis presented by Cansu Kücükköse Freiburg im Breisgau July 2020 176 Seiten Illustrationen, Diagramme 30 cm txt rdacontent n rdamedia nc rdacarrier Enthält Zeitschriftenaufsätze Dissertation Albert-Ludwigs-Universität Freiburg 2020 (DE-588)4113937-9 Hochschulschrift gnd-content B:DE-101 application/pdf https://d-nb.info/1225837421/04 Inhaltsverzeichnis DNB Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=032850133&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis 1\p aepgnd 0,08328 20210209 DE-101 https://d-nb.info/provenance/plan#aepgnd |
| spellingShingle | Kücükköse, Cansu Proteolytic control of mitochondrial protein biogenesis |
| subject_GND | (DE-588)4113937-9 |
| title | Proteolytic control of mitochondrial protein biogenesis |
| title_auth | Proteolytic control of mitochondrial protein biogenesis |
| title_exact_search | Proteolytic control of mitochondrial protein biogenesis |
| title_exact_search_txtP | Proteolytic control of mitochondrial protein biogenesis |
| title_full | Proteolytic control of mitochondrial protein biogenesis presented by Cansu Kücükköse |
| title_fullStr | Proteolytic control of mitochondrial protein biogenesis presented by Cansu Kücükköse |
| title_full_unstemmed | Proteolytic control of mitochondrial protein biogenesis presented by Cansu Kücükköse |
| title_short | Proteolytic control of mitochondrial protein biogenesis |
| title_sort | proteolytic control of mitochondrial protein biogenesis |
| topic_facet | Hochschulschrift |
| url | https://d-nb.info/1225837421/04 http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=032850133&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
| work_keys_str_mv | AT kucukkosecansu proteolyticcontrolofmitochondrialproteinbiogenesis |
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