Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis:
Gespeichert in:
| 1. Verfasser: | |
|---|---|
| Format: | Buch |
| Sprache: | English |
| Veröffentlicht: |
2003
|
| Schlagworte: | |
| Online-Zugang: | Inhaltsverzeichnis |
| Beschreibung: | München, Techn. Univ., Diss., 2003 |
| Beschreibung: | VI, 130 S. Ill., graph. Darst. |
Internformat
MARC
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| 100 | 1 | |a Mayerhofer, Peter Uli |d 1971- |e Verfasser |0 (DE-588)124989489 |4 aut | |
| 245 | 1 | 0 | |a Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis |c Peter Uli Mayerhofer |
| 264 | 1 | |c 2003 | |
| 300 | |a VI, 130 S. |b Ill., graph. Darst. | ||
| 336 | |b txt |2 rdacontent | ||
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Datensatz im Suchindex
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| adam_text |
TABLE
OF
CONTENTS
ABSTRACT
.
III
ZUSAMMENFASSUNG
.
V
CHARIER
I:
INTRODUCTION
.
1
1
PEROXISOMES:
MORPHOLOGY
AND
METABOLIC
FUNCTIONS
.
2
2
PEROXISOMAL
DISEASES
.
5
2.1
X-LINKED
ADRENOLEUKODYSTROPHY:
A
PEROXISOMAL
SINGLE
ENZYME
DEFICIENCY
.
6
2.2
PEROXISOMAL
BIOGENESIS
DISORDERS
.
8
3
THE
PEROXISOMAL
BIOGENESIS
.
10
3.1
TARGETING
AND
IMPORT
OF
MATRIX
ENZYMES
.
13
32
PEROXISOMAL
MEMBRANE
BIOGENESIS
.
17
3.3
PEX3
AND
PEX19,
PEROXINS
EXPECTED
TO
BE
INVOLVED
IN
THE
EARLY
STEPS
OF
PEROXISOMAL
BIOGENESIS
.
20
CHAPTER
II:
AIMS
OF
THE
THESIS
.
22
CHAPTER
III:
RESULTS
AND
DISCUSSION
.
24
1
PEX19
INTERACTS
WITH
A
VARIETY
OF
PEROXISOMAL
MEMBRANE
PROTEINS
.
25
2
CHARACTERIZATION
OF
PEX19
SPLICE
VARIANTS:
FUNCTIONAL
DIVERSITY
AND
NEW
INSIGHTS
IN
THE
ROLE
OF
POSTTRANSLATIONAL
FAMESYLATION
.
27
3
PROPOSED
BIOLOGICAL
FUNCTIONS
OF
PEX19
.
30
4
PEX3,
A
KEY
FACTOR
IN
EARLY
HUMAN
PEROXISOME
SYNTHESIS
.
32
5
TWO-PATH
MODEL
FOR
PEROXISOME
MEMBRANE
SYNTHESIS
IN
MAMMALIAN
CELLS
.
35
6
FURTHER
PERSPECTIVES:
IDENTIFICATION
OF
THE
ORIGIN
OF
THE
ENDOMEMBRANE
TEMPLATE.
37
REFERENCES
.
38
APPENDIX
1:
FULL
LENGTH
CDNA
CLONING,
PROMOTER
SEQUENCE,
AND
GENOMIC
ORGANIZATION
OF
THE
HUMAN
ADRENOLEUKODYSTROPHY
RELATED
(ALDR)
GENE
FUNCTIONALLY
REDUNDANT
TO
THE
GENE
RESPONSIBLE
FOR
X-LINKED
ADRENOLEUKODYSTROPHY
.
50
APPENDIX
2:
HUMAN
ADRENOLEUKODYSTROPHY
PROTEIN
AND
RELATED
PEROXISOMAL
ABC
TRANSPORTERS
INTERACT
WITH
THE
PEROXISOMAL
ASSEMBLY
PROTEIN
PEX19P
.
63
APPENDIX
3:
TWO
SPLICE
VARIANTS
OF
HUMAN
PEX19
EXHIBIT
DISTINCT
FUNCTIONS
IN
PEROXISOMAL
ASSEMBLY
.
77
APPENDIX
4:
GENOMIC
ORGANIZATION,
EXPRESSION
ANALYSIS,
AND
CHROMOSOMAL
LOCALIZATION
OF
THE
MOUSE
PEX3
GENE
ENCODING
A
PEROXISOMAL
ASSEMBLY
PROTEIN
.
90
T
ABLE
OF
C
ONTENTS
II
APPENDIX
5:
THE
HUMAN
PEX3
GENE
ENCODING
A
PEROXISOMAL
ASSEMBLY
PROTEIN:
GENOMIC
ORGANIZATION,
POSITIONAL
MAPPING,
AND
MUTATION
ANALYSIS
IN
CANDIDATE
PHENOTYPES
.
100
APPENDIX
6:
DEFECTIVE
PEROXISOME
MEMBRANE
SYNTHESIS
DUE
TO
MUTATIONS
IN
HUMAN
PEX3
CAUSES
ZELLWEGER
SYNDROME,
COMPLEMENTATION
GROUP
G
.
112
ABBREVIATIONS
.
125
DANKSAGUNG
.
126
LIST
OF
PUBLICATIONS
.
127
CURRICULUM
VITAE
.
130 |
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| discipline | Biologie |
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| id | DE-604.BV017463548 |
| illustrated | Illustrated |
| indexdate | 2024-08-14T00:57:08Z |
| institution | BVB |
| language | English |
| oai_aleph_id | oai:aleph.bib-bvb.de:BVB01-010521934 |
| oclc_num | 76638335 |
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| owner_facet | DE-91 DE-BY-TUM DE-M49 DE-BY-TUM DE-12 |
| physical | VI, 130 S. Ill., graph. Darst. |
| publishDate | 2003 |
| publishDateSearch | 2003 |
| publishDateSort | 2003 |
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| spelling | Mayerhofer, Peter Uli 1971- Verfasser (DE-588)124989489 aut Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis Peter Uli Mayerhofer 2003 VI, 130 S. Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier München, Techn. Univ., Diss., 2003 Biogenese (DE-588)4006799-3 gnd rswk-swf Membran (DE-588)4038571-1 gnd rswk-swf Peroxisom (DE-588)4128263-2 gnd rswk-swf Mensch (DE-588)4038639-9 gnd rswk-swf (DE-588)4113937-9 Hochschulschrift gnd-content Mensch (DE-588)4038639-9 s Peroxisom (DE-588)4128263-2 s Membran (DE-588)4038571-1 s Biogenese (DE-588)4006799-3 s DE-604 DNB Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=010521934&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Mayerhofer, Peter Uli 1971- Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis Biogenese (DE-588)4006799-3 gnd Membran (DE-588)4038571-1 gnd Peroxisom (DE-588)4128263-2 gnd Mensch (DE-588)4038639-9 gnd |
| subject_GND | (DE-588)4006799-3 (DE-588)4038571-1 (DE-588)4128263-2 (DE-588)4038639-9 (DE-588)4113937-9 |
| title | Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis |
| title_auth | Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis |
| title_exact_search | Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis |
| title_full | Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis Peter Uli Mayerhofer |
| title_fullStr | Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis Peter Uli Mayerhofer |
| title_full_unstemmed | Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis Peter Uli Mayerhofer |
| title_short | Functional characterization of the human peroxins PEX3 and PEX19, proteins essential for early peroxisomal membrane biogenesis |
| title_sort | functional characterization of the human peroxins pex3 and pex19 proteins essential for early peroxisomal membrane biogenesis |
| topic | Biogenese (DE-588)4006799-3 gnd Membran (DE-588)4038571-1 gnd Peroxisom (DE-588)4128263-2 gnd Mensch (DE-588)4038639-9 gnd |
| topic_facet | Biogenese Membran Peroxisom Mensch Hochschulschrift |
| url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=010521934&sequence=000001&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
| work_keys_str_mv | AT mayerhoferpeteruli functionalcharacterizationofthehumanperoxinspex3andpex19proteinsessentialforearlyperoxisomalmembranebiogenesis |