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PREFACE Molecular biologists' concept of cells is a "biological container" where important molecules float and interact with one another more or less randomly. However, a close view of this "bag" reveals an astonishing net of fibrous proteins crossing throughout the cytoplasm: the cytoskeleton. This structure is composed of three different elements: actin microfilaments, tubulin microtubules, and a third class denoted, due to their size, intermediate filaments (IF). This last network is built by...
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MOLECULAR BIOLOGY INTELLIGENCE UNIT Landes Bioscience, a bioscience publisher, is making a transition to the internet as Eurekah.com. INTELLIGENCE UNITS Biotechnology Intelligence UnitMedical Intelligence UnitMolecular Biology Intelligence UnitNeuroscience Intelligence Unit Tissue Engineering Intelligence Unit The chapters in this book, as well as the chapters of all of the five Intelligence Unit series, are available at our website. EUREKAH.COM ISBN 0-387-33780-6 [weiter lesen] |
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CONTENTS Prefaceix 1.Desmin and Other Intermediate Filaments in Normal and Diseased Muscle 1 Denise Paulin and Zhigang Xue Desmin in Mature Skeletal and Heart Muscle Cells 1 Desmin and Other IF Proteins in Different Muscles 2 Functions of Desmin in Striated Muscles 4 Function of Desmin in Smooth Muscles 5 Gene Regulation 5 Desmin-Related Myopathies (DRM)6 2.Intermediate Filaments in Astrocytes in Health and Disease 10 Milos Pekny and Ulrika Wilhelmsson IFs in Astroglial Cells during Development 10 GFAP and Its Regulation 11 Vimentin and Nestin and Their Regulation 13 Formation of IFs in Astroglial Cells 13 Astrocyte IFs, CNS Trauma and Regeneration 16 Astrocyte IFs and Resistance to Mechanical Stress 22 Astrocyte IFs, Osmotic Stress and Ischemia/Hypoxia 25 Astrocyte IFs, Cell Proliferation and Tumorigenesis 25 GFAP Mutations and Alexander Disease 26 3.Neuronal Intermediate Filaments and Neurodegenerative Diseases 35 Gee Y. Ching and Ronald K.H. Liem Neuronal Intermediate Filament Proteins 36 Neuronal IFs in Neurodegenerative Diseases 38 4.Neurofilaments: Phosphorylation and Signal Transduction 52 Sashi Kesavapany, Richard H. Quarles and Harish C. Pant Neurofilaments Are Members of the Intermediate Filament Family 53 Neurofilaments and Axonal Caliber 55 Phosphorylation of Neurofilaments 55 Neurofilaments and Their Associated Kinases 56 Neurofilament Tail Domain Phosphorylation 57 Axonal Transport of Neurofilaments 58 Phosphorylation of NFs and Disease 59 Neurofilaments and Signal Transduction Pathways 60 5.Keratin Intermediate Filaments and Diseases of the Skin 74 E.Birgitte Lane Epidermal Blistering Caused by Keratin Mutations 74 Candidate Genes in Other Epithelia 75 From Candidate Genes to "Candidate Diseases"77 Supramolecular Stability versus Molecular Flux 77 Keratin Filaments as Tensile Structures 78 Significance of Keratin Role in Skin Fragility Disorders 79 6.The Keratin K 6 Minifamily of Genes 83 Manuel Navarro Conservation of Sequence in the K 6 Polypeptides 84 Expression of the K 6 Genes 87 Expression and Partners 89 Functional Considerations 89 7.Transcriptional Regulation of Keratin Gene Expression 93 Miroslav Blumenberg Transcriptional Regulation of Keratin K 5 Expression 93 Regulation of K 1495 Regulation of K 1596 Regulation of K 1 and K 1096 Regulation of K 4 and K 1397 Regulation of K 3 and K 1298 Regulation of Hair Keratins 99 Regulation of Keratin Gene Expression by Hormones and Vitamins 99 Regulation of Keratin Gene Expression by Growth Factors and Cytokines 101 Regulation of Small Keratins, K 7, K 8, K 18 and K 19101 Specific Transcription Factors Implicated in Regulation of Transcription of ... Conclusions and Future Directions 105 8.Simple Epithelial Keratins: Expression, Function and Disease 110 M. Lianos Casanova, Ana Bravo and Jose L. Jorcano Suggested Functions of Simple Epithelial Keratins 111 Simple Epithelial Keratin Proteins and Disease 114
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Index Symbols 14-3-3 41, 113, 126, 141 A α-internexin 35-37, 39, 42, 44, 54 αB-crystallin 125 Actin 1, 41, 43, 53, 66, 77-79, 111, 132, 133 Aggregation 6, 7, 26, 40, 44, 45, 56, 59, 60, 75, 77, 78, 111, 120, 121, 123, 124, 13... Akt 136-141 Alcoholic steatohepatitis (ASH) 120, 121, 123-126 Alexander disease 26, 27, 79 Amyotrophic lateral sclerosis (ALS) 35, 38-41, 43-45, 51, 52, 59, 60, 79 API 12, 13, 88, 89, 93-96, 98, 100-104 AP 2 88, 89, 93-99, 101, 103, 104 Apoptosis 114, 115, 125-127, 142 Astrocyte 3, 10-27 Axon 17, 20, 23, 35, 37-39, 41-45, 52-56, 58-61, 63, 64, 67, 69 Axonal transport 35, 39, 41-45, 52, 55, 58, 59 BBasal layer 74, 75, 83, 90, 94-97, 132, 134, 138, 141, 142 Blistering 74, 76, 77, 89, 114 CC/EBP 88, 89, 96, 98, 104 Calmodulin-dependent protein kinase (CAMK) 42, 52, 57 Cancer 97, 103, 110, 115, 116, 121, 122, 132 Casein kinase I/II (CKI/II) 42, 52, 57 Caspase 114, 120, 127 CBP/p 300 95, 102, 104 Cdk 4 45 Cdk 5 42, 43, 45, 52, 57-60, 62-65, 67-69 Charcot-Marie-Tooth disease 35, 39 Cholestasis 120, 121, 123 Cirrhosis 77, 120, 121, 127 Copper storage 121 Cyclin 42, 57, 97, 134, 138, 141 Cytoskeleton 1, 4-6, 25, 35, 36, 41, 44, 52-54, 56, 58, 64, 67, 74, 77-80, 83, 90, 11... DDelta 99 Desmin 1-7, 56, 79 Desmin-related myopathies (DRM) 1, 5-7 Desmosomes 78, 79, 113, 124, 139, 140 - 3, 5-diethoxycarbonyl-1, 4-dihydrocollidine (DDC) 122-126 Differentiation 4, 6, 12, 20, 22, 41, 53-55, 65, 75, 76, 78, 83, 95-100, 105, 110, 12... Disease 1, 6, 10, 16, 26, 27, 35, 38-41, 43-45, 52, 59, 60, 69, 74, 75, 77, 79, 80, 8... Dorsal root ganglia neuron (DRG) 54, 55, 64-66, 69 Dynein 44, 45, 58, 59 EEnhanced green fluorescent (EGF) 22, 23, 40, 61, 88, 89, 100, 101 Enhancer 5, 6, 13, 88, 94, 99, 102, 103 Epidermis 22, 75, 76, 83, 90, 94-97, 99-101, 103, 111, 114, 115, 121, 131, 132, 134, ... Epidermolysis bullosa simplex (EBS) 74-78 Epithelia 74, 75, 76, 77, 78, 79, 80, 83, 94, 95, 96, 97, 101, 102, 103, 105, 110, 11... Epithelial 74, 75, 76, 77, 78, 79, 83, 93, 94, 95, 96, 97, 98, 99, 102, 105, 110, 111... ERK 42, 52, 57, 58, 60, 61, 64-69, 126, 141 Ets 95, 97, 102, 104 FFas 113, 114, 126 GGene expression 11, 35, 40, 45, 93-103, 121, 131 Glia 10, 12, 16, 23, 63, 69 Glial fibrillary acidic protein (GFAP) 3, 10-27, 56, 79 Glucocorticoid receptor (GR) 96, 100, 104 Glycogen synthase kinase-3 (GSK 3) 42, 52, 57 Griseofulvin (GF) 122, 123, 126 HHepatocyte 77, 111-114, 120-125 HOX 99, 104 IIchthyosis bullosa of Siemens 76 Inducible expression 87, 88 Inflammatory bowel disease 77, 115 Integrin 52, 62, 64, 132, 141 Intermediate filament (IF) 1-7, 10, 13-20, 22, 23, 25, 28, 35-45, 52-56, 74, 75, 77-8... JJun NH 2 -terminal kinase (JNK) 113, 114, 126 KKeratin (K) 1, 3, 22, 35, 74-80, 83-90, 93-105, 110-116, 120-127, 131-142 Keratinocyte 74, 75, 78, 79, 83, 84, 89, 94-101, 103, 115, 121, 131, 132, 134, 136-13... Kinase 4, 13, 17, 35, 41-43, 45, 52, 56-65, 67-69, 86, 113, 114, 122, 125, 126, 134, ... Kinesin 4, 44, 58, 59 Knock-out mice 4, 6, 60, 77, 89, 95, 111, 120-122, 125, 141 Krox 5, 6 Kruppel-like transcription factor (KLF) 97, 98, 103, 104 KSP 36, 37, 39, 40, 42, 43, 52-55, 57-59, 61, 62, 64, 67 LLewy body 38, 52, 59 Liver 77, 111-115, 120-124, 126, 127 Locus controlling region (LCR) 102, 103 Lymphoid enhancer factor 1 (LEF-1) 99 Lysine-serine-proline 37, 53, 54, see also KSP MMallorybody 77, 111, 120-126 Meesman corneal dystrophy (MCD) 76 Mitochondria 1, 4, 41, 42, 43, 44, 59, 121, 139, 140 Mitogen-activated protein kinase (MAP kinase) 43, 52, 57, 59-61, 63, 64 Monilethrix 76 Motif 54, 55, 58, 75-77, 96-99, 105 Muscle 1-7, 38, 39, 60, 79, 80 Myelin associated glycoprotein (MAG) 52, 63-67, 69 MyoD 5, 6 Myopathy 1, 5-7, 79 NNestin 1, 3, 4, 10, 13-16, 36, 37, 53, 54 Neurodegenerative disease 16, 27, 35, 38-41, 43, 45, 52, 59, 69 Neurofilament (NF) 13, 35-45, 52-69, 79, 95, 96 Neurons 10-12, 17, 22, 23, 26, 35-45, 52-56, 58-61, 63-67, 69, 112 NFKB 13, 95, 96,... Nonalcoholic steatohepatitis (NASH)120-122, 124, 126, 127 Notch 99, 142 OOMIM 75-77, 79, 89, 138 Pp 62 121-125 Pachyonychia congenita (PC) 76, 86, 89, 90 Pancreas 103, 110-115, 138 Paranemin 1, 6 Parkinsons disease 38, 39, 52, 59 PAX 98, 104 Peripherin 35-42, 45, 53, 54, 60 Phosphorylation 13, 15, 35, 37, 39, 41-45, 52-69, 77, 78, 101, 113, 114, 122, 125-127... Placenta 111 Plectin 6 Polarity 77, 112, 113 POU 95 Proliferation 10, 25, 26, 54, 76, 83, 87, 95, 97, 101, 110, 116, 120, 121, 126, 132, ... Promoter 6, 11-13, 39, 87, 88, 93-99, 101-103, 114-116, 138, 139 Protein kinase A (PKA) 13, 42, 52, 56, 57 Protein kinase C (PKC) 13, 42, 56, 57, 113 Protein misfolding 120, 124, 125 Purkinje 2, 35, 39, 42 RRARE 88, 89 Retinoblastoma (Rb) 45, 99, 134, 136, 138, 139, 141, 142 Retinoic acid 62, 83, 93, 94, 96, 97, 99, 100, 102, 103 Rod domain 36, 41, 53, 54, 56, 76, 86, 111 SSignal transduction 52, 60, 62, 67-69, 80, 113, 116, 138, 139 Skin 74-77, 79, 80, 83, 89, 100, 101, 103, 111, 115, 116, 131, 136, 138, 139, 141 Skn-1 95, 104 Smooth muscle 1-3, 5 Spl 5, 6, 13, 93, 94, 97-99, 101-104 STAT 13 Steatocystoma multiplex 76, 89 Steatohepatitis 120-123 Superoxide dismutase (SOD) 35, 39, 43-45, 60 Syncoilin 3, 4, 6 Synemin 1, 3-6, 10, 15, 16 TT 3 R 99, 100, 104 TATA box 88, 97, 99, 102, 103 tau 43, 53, 59, 64, 66, 67, 68 Thymus 142 Thyroid hormone 93, 94, 96, 99, 100 Transcription factor 12, 88, 93-98, 100-105, 115 Transfection 26, 44, 56, 58, 61, 94-98, 100, 115, 122, 132, 134, 136 Transforming growth factor (TGF) 100, 101, 113 Transgenic mice 13, 26, 35, 37-40, 42-45, 55, 56, 60, 87, 94, 95, 99, 100, 102, 103, ... Transitional cell carcinoma (TCC) 98, 115 Transport 13, 25, 35, 37, 39, 41-45, 52, 55, 56, 58-60, 113 Tubulin 43, 57, 60, 66, 77, 79, 132, 133 Tumor 25-27, 97, 101, 110, 114-116, 121, 126, 138-141 Tumor necrosis factor (TNF) 101, 111, 114, 115, 121, 126, 141 UUbiquitin 40, 121, 122, 124, 125 VVimentin 1-6, 10, 13-17, 23, 26, 36, 37, 53, 54, 56, 77, 115, 132, 133 Vitamin D 3 99 WWhite sponge naevus (WSN) 76 Whn 97, 104 Wound healing 18, 83, 86, 89, 90, 96, 121, 132
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