11 research outputs found
Revisiting the persistency and volatility of the firm's R&D investment based on its technological capabilities
νμλ
Όλ¬Έ (μμ¬)-- μμΈλνκ΅ λνμ : νλκ³Όμ κΈ°μ κ²½μΒ·κ²½μ Β·μ μ±
μ 곡, 2014. 2. μ΄μ λ.κ·Έ λμ μ νμ κ΄μ μμ μ°κ΅¬κ°λ°ν¬μ μ΅μ ν¬μλ, κ²°μ μμΈμ λΆμμ΄ λ§μ΄ μ΄λ£¨μ΄μ§κ³ μ‘μ§λ§, λνμ κ΄μ μμ μ°κ΅¬κ°λ°ν¬μμ λν λΆμμ μλμ μΌλ‘ λ―Έλ―Ένλ€. μ°κ΅¬κ°λ°ν¬μλ κ·Έ λμμ νλ κ²½ν, μ΄μ κΈ°μ ν¬μ μ λ μ μν₯μ λ§μ΄ λ°κΈ° λλ¬Έμ λνμ κ΄μ μμ λΆμνλ κ²μ΄ μ€μνλ€.
λνμ κ΄μ μμ μ§νλ μ νμ°κ΅¬μ μνλ©΄, μ°κ΅¬κ°λ°ν¬μλ μ§μμ±μ 보μΈλ€. μ¦, κΈκΈ°μ μ°κ΅¬κ°λ°ν¬μλ μ κΈ°μ ν¬μ μ λλ₯Ό μ μ§νλ €κ³ νλ€. μ§μμ±μ 보μ΄λ μ΄μ λ μ°κ΅¬κ°λ°ν¬μμ λμ μ‘°μ λΉμ©κ³Ό 맀λͺ°λΉμ©μ μΈ μ±κ²© λλ¬Έμ΄λ€. μ΄λ¬ν λΉμ©μ μ΅μννκΈ° μν΄ κΈ°μ
μ 보μ νκΈμ ν¬μ
νμ¬μ μ°κ΅¬κ°λ°ν¬μ λ³λμ μ€μ΄κ³ μ§μνλ €κ³ λ
Έλ ₯νλ€. νμ§λ§ μ΄λ° λ°°κ²½μλ λΆκ΅¬νκ³ λͺλͺ μ€μ¦μ°κ΅¬μμ μ°κ΅¬κ°λ°ν¬μλ 맀λ
λ³λμ±μ 보μΈλ€κ³ 보μλ€. κ·Έ μ΄μ λ μ°κ΅¬κ°λ°ν¬μλ λ΄λΆνκΈνλ¦ λ³λμ λ°λΌ λ―Όκ°νκ² λ°μνκΈ° λλ¬Έμ΄λ€.
λνμ κ΄μ μμ μ°κ΅¬κ°λ°ν¬μμ κ΄ν μ νμ°κ΅¬λ€μ λ
Όμκ° μλ‘ μΌμΉλμ§ μκ³ μλ€. λ³Έ μ°κ΅¬μμλ μ νμ°κ΅¬μμ μλ‘ μλ°λ λ
Όμλ₯Ό 보μ΄λ μ΄μ λ₯Ό μ€μ¦μ μΌλ‘ λΆμνμλ€. λ³λ μΆ©κ²©μ΄ μ£Όμ΄μ§λ€λ©΄, κΈ°μ
κ° μλ‘ λμΌνκ² λ°μνκ³ μ, μ 좩격 λ³λμ λμΉμ μΌλ‘ λ°μνλ€λ μ μ νμμ μ΄λ£¨μ΄μ§ μ νμ°κ΅¬μ νκ³λ₯Ό 보μνμ¬ λΆμνμλ€. μ΄λ₯Ό μν΄ κΈ°μ
κ³ μ μ μ°κ΅¬κ°λ°νλμ κ²°μ μ§λ κΈ°μ μλμ ν΅ν΄ κΈ°μ
κ° μ΄μ§μ±μ κ³ λ €νμμΌλ©°, μμ λ³λκ³Ό μμ λ³λμ λ°λΌ κΈ°μ μλμ μν μ°¨μ΄κ° λ€λ₯Ό κ²μ΄λΌλ κ°μ€ νμμ μ€μ¦λΆμμ μννμλ€.
μ€μ¦λΆμμ μνν κ²°κ³Ό, κΈ°μ
맀μΆμ‘μ΄ μ λ
λλΉ μ¦κ°νλ μμ 좩격 νμλ κΈ°μ μλμ νκΈνλ¦ν¨κ³Όλ₯Ό μ¦νμμΌ μ°κ΅¬κ°λ°ν¬μλ₯Ό λ³λνλλ‘ νμλ€. μ΄μ λ°ν΄ 맀μΆμ‘μ΄ μ λ
λλΉ κ°μνλ μμ 좩격 νμλ κΈ°μ μλμ νκΈνλ¦ν¨κ³Όλ₯Ό μμμμΌ μ°κ΅¬κ°λ°ν¬μλ₯Ό μ§μνλλ‘ νμλ€. μ΄λ¬ν κ²°κ³Όλ κΈ°μ‘΄ μ°κ΅¬λ€μ²λΌ μ§μμ±κ³Ό λ³λμ±μ΄ μ΄λΆλ²μ μΌλ‘ λ립λλ κ²μ΄ μλ νΉμ μν© νμμ λ λ€ λνλλ κ²μ νμΈνλ€λ μ μμ μμκ° μλ€. λν μμ 좩격 νμμ μμμ μΌλ‘ ν¬μλ₯Ό μ μ§ν μ μκ² νλ κΈ°μ μλμ κ°νμν€κ² νλ μ μ±
μ μ§μμ΄ νμνλ€λ μμ¬μ μ λμΆν μ μλ€.1. μλ‘
1.1 μ°κ΅¬λ°°κ²½ λ° λͺ©μ
2. μ΄λ‘ μ λ°°κ²½
2.1 μ°κ΅¬κ°λ°ν¬μ μ§μμ±
2.1.1 μ°κ΅¬κ°λ°ν¬μ μ§μμ± μ΄λ‘ μ κ·Όκ±°
2.1.2 R&D smoothing: μ°κ΅¬κ°λ°ν¬μλ₯Ό μ§μνκΈ° μν λ
Έλ ₯
2.1.3 μ°κ΅¬κ°λ°ν¬μ μ§μμ± μ μΈ μ°¨μ΄
2.2 μ°κ΅¬κ°λ°ν¬μμ λ³λμ±
2.2.1 R&D financing: μ°κ΅¬κ°λ°ν¬μμ λ΄λΆνκΈνλ¦
2.2.2 Financial constraints: μ°κ΅¬κ°λ°ν¬μμ νκΈμ μ½
2.3 μ°κ΅¬κ°λ°ν¬μ μ§μμ±κ³Ό λ³λμ±
2.4 κΈ°μ μλ
2.4.1 κΈ°μ μλ μ μ λ° λ²μ£Ό
2.4.2 κΈ°μ μλμ΄ μ°κ΅¬κ°λ°ν¬μ νλμ λ―ΈμΉλ μν₯
2.4.3 κΈ°μ μλμ΄ μ°κ΅¬κ°λ°ν¬μ νμ΅μ λ―ΈμΉλ μν₯
3. μ°κ΅¬λͺ¨ν λ° μ°κ΅¬λ°©λ²
3.1 μ°κ΅¬κ°μ€
3.2 μ°κ΅¬λͺ¨ν
3.3 μλ£
3.4 λ³μμ μ
3.4.1 λ
립λ³μ: κΈ°μ μλ μΈ‘μ
3.4.2 ν΅μ λ³μ
4. λΆμκ²°κ³Ό
4.1 κΈ°μ΄λΆμ
4.2 λΆμκ²°κ³Ό
4.3 λΆμκ²°κ³Ό κ³ μ°°
5. κ²°λ‘ λ° μμ¬μ
μ°Έ κ³ λ¬Έ ν
λΆλ‘ 1 : μ°κ΅¬κ°λ°ν¬μ λ³λ μ λ μΈ‘μ λ°©λ²
λΆλ‘ 2 : μ΄λΆμ°μ±μ κ³ λ €ν κ³ μ ν¨κ³Όλͺ¨ν κ²°κ³Ό
AbstractMaste
νμ νλμ μ§μμ± κ΄μ μμ κ³ μ°°ν νμ μ κΈ°μ μ±μ₯ κΈ°μ¬ν¨κ³Ό λΆμ
νμλ
Όλ¬Έ (λ°μ¬)-- μμΈλνκ΅ λνμ : 곡과λν νλκ³Όμ κΈ°μ κ²½μΒ·κ²½μ Β·μ μ±
μ 곡, 2018. 8. μ΄μ λ.νμ μ κΈ°μ
μ±μ₯μ μλλ ₯μ΄λ€. μ΄μ κ΄λ ¨ν λ€μμ μ νμ°κ΅¬κ° μ§νλμμ§λ§, μ¬μ ν νμ€μμ λνλλ κΈ°μ
μ±μ₯μ λν νμ μ κΈ°μ¬ν¨κ³Όλ₯Ό μ΄ν΄νλλ° νκ³κ° μλ€. μλ₯Ό λ€μ΄, νκ΅κΈ°μ
λ€μ νμ νλμ΄ κΎΈμ€ν μ¦κ°ν¨μλ λΆκ΅¬νκ³ μ΅κ·Ό κΈ°μ κ²½μλ ₯ λ° μ±κ³Όκ° μ§μμ μΌλ‘ νλ½(μ 체)νλ μ΄μ λ₯Ό μ€λͺ
νμ§ λͺ»νλ€. κ·Έ μ΄μ λ κ·Έ λμ μ°κ΅¬μμ νμ νλμ μμ μΈ‘λ©΄μμλ§ λ
Όμκ° μ΄λ€μ‘κΈ° λλ¬Έμ΄λ€. μ¦, κΈ°μ
μ νμ νλμ μμ μ°¨μ΄κ° μ§μμΆμ μ λλ₯Ό κ²°μ νκ³ , κ·Έ κ²°κ³Ό κΈ°μ
κ²½μλ ₯(μ±μ₯) μ°¨μ΄λ₯Ό κ²°μ νλ€λ μ μ νμμλ§ λ
Όμκ° μ΄λ€μ‘λ€.
νμ§λ§ μ§μμ μλ³Έκ³Ό λ€λ₯΄κ² μμ λ
Έλ ₯λ§μΌλ‘ μΆμ λλ κ²μ΄ μλλ€. μ§μμ μ₯κΈ°κ° μνμ°©μ€λ₯Ό ν΅ν κΎΈμ€νκ³ μ§μμ μΈ λ
Έλ ₯μ κ²°κ³Όλ‘ μΆμ λλ€. κ·Έλ¬λ―λ‘ νμ νλμ μ΄ν΄νλ μλ‘μ΄ μΆμΌλ‘ κΈ°μ
μ΄ μΌλ§λ μ§μμ μΌλ‘ λ
Έλ ₯, μΆμ νκ³ μλμ§μ λν λ
Όμκ° νμνλ€. μ΄λ¬ν κ΄μ νμμμ μ°κ΅¬λ λΆμ‘±νλ€.
λ³Έ μ°κ΅¬λ νμ νλμ μ§μμ± κ΄μ μμ νμ μ΄ κΈ°μ
μ±μ₯μ λ―ΈμΉλ κΈ°μ¬ν¨κ³Όλ₯Ό μ€μ¦μ μΌλ‘ λΆμνμλ€. νκ΅ μ μ‘°κΈ°μ
μ λμμΌλ‘ R&D, νΉν λ° μ ν μ 보λ₯Ό ν¬κ΄νλ κ³ μ μ ν¨λ λ°μ΄ν° μ
μ ꡬμΆνμμΌλ©°, μ€μ¦λΆμμ μ§ννμλ€. μ΄λ₯Ό ν΅ν΄ κΈ°μ
κ²½μμ°μμ μμ²μΌλ‘ κΎΈμ€νκ³ μ§μμ μΈ νμ νλμ΄ μ€μνλ€λ μ μ κ·λͺ
νμλ€.
ꡬ체μ μΌλ‘, 3μ₯μμλ R&Dν¬μ μ§μμ±μ μ€μμ±μ κ·λͺ
νμλ€. μΌμ κΈ°κ° λμμ λμΌν μ λμ R&Dν¬μλ₯Ό ν κΈ°μ
κ΅° λ΄μμ μΌλΆ κΈ°μ
μ 맀λ
μΌμ μ λλ‘ ν¬μ(R&Dμ§μμ±)νλ λ°λ©΄μ μΌλΆλ 맀λ
λ³λ(R&Dλ³λμ±) νλ μ΄μ§μ±μ΄ κ΄μ°°λλ€. λΆμκ²°κ³Όμ μνλ©΄, R&Dν¬μλ₯Ό μ§μνλ κΈ°μ
μΌμλ‘ λμ μ±μ₯λ₯ μ 보μΈλ€. νΉν, R&Dν¬μκ° μ¦κ°ν μλ‘ λμ μ±μ₯λ₯ μ 보μ΄λ κ²½μ°λ R&Dν¬μλ₯Ό μ§μνλ κΈ°μ
κ΅°μμλ§ λνλλ€. μ΄μ λ°ν΄, R&Dκ° λ³λνλ κΈ°μ
κ΅°μμ λμ R&Dν¬μλ μ€νλ € κΈ°μ
μ±μ₯μ μ ν΄νλ€. κ·Έλ¬λ―λ‘ R&Dν¬μκ° μ λλΌλ μ§μμ μΌλ‘ νλ κΈ°μ
μ΄ R&Dν¬μκ° λ§λλΌλ 맀λ
λ³λνλ κΈ°μ
λ³΄λ€ λ λμ μ±μ₯λ₯ μ 보μΈλ€. μ΄λ¬ν κ²°κ³Όλ κΈ°μ
λ΄αμΈλΆ νκ²½μ΄ λ³νλλΌλ ν λ² μ€μ λ νμ μ λ΅μ μ₯κΈ°μ μΈ μλͺ©μ κ°μ§κ³ κ³ μνλ λ
Έλ ₯μ΄ μ€μν¨μ μμ¬νλ€.
4μ₯μμλ κΈ°μ
λ΄ μ§μκΈ°λ° μ§μμ μ€μμ±μ κ·λͺ
νμλ€. λΆμκ²°κ³Όμ μνλ©΄, ν λΆμΌμμ μ§μμ μΈ κ²½νμ μΆμ νλ κΈ°μ
μΌμλ‘ λμ μ±μ₯μ νλ€. νμ§λ§ κ²½νμ μ λμ λ°λΌμ μ§μμΆμ μ μν νμ΅μ λ΅μ λ³νν΄μΌ νλ€. ꡬ체μ μΌλ‘, κ²½νμ΄ μλ μνμμλ λ°λ³΅μ μΈ νμ΅μ λ΅(μ€ν μ΅μν)μ κ³ μνλ©΄μ μ§μμ μΆμ νλ €λ λ
Έλ ₯μ΄ μ€μνλ€. νμ§λ§ κ΄λ ¨ μ§μμ΄ μΆμ λ μλ‘, λ€μν μ§μκ³Όμ μλ‘μ΄ κ²°ν©μ μλ(μ€ν)νλ νμ΅μ λ΅μ΄ μ€μνλ€. μ΄λ₯Ό ν΅ν΄ κΈ°μ
μ ν λΆμΌμμμ μ§μμ μ§μμ μΌλ‘ μΆμ νλ, λ€μν λ°©μμΌλ‘μ μλλ₯Ό ν΅ν μ§μμΆμ μ λ΅μ΄ νμν¨μ μ΄ν΄ν μ μλ€.
λ§μ§λ§ 5μ₯μμλ κΈ°μ
μ μ ν κ΅¬μ± λλΉ μ§μ ꡬμ±μ μλμ μ§μμ μ€μμ±μ κ·λͺ
νμλ€. κΈ°μ
λ΄ μ§μκ³Ό μ νμ μνΈμν₯μ μ£Όκ³ λ°μΌλ©° λ°μ , μ§ννλλ°, μ΄λ¬ν κ΄κ³λ₯Ό μ΄ν΄λ³΄κ³ , μ΄λ ν μ λ΅μ΄ κΈ°μ
μ±μ₯μ ν¨μ¨μ μΈμ§ νμ
νμλ€. λΆμκ²°κ³Όμ μνλ©΄, ν΅μ¬μ§μμ 보μ νλ©΄μ, λ€μν μ νκ΅°μ κ°μ§ κΈ°μ
μΌμλ‘ λμ μ±μ₯λ₯ μ 보μΈλ€. ν΅μ¬μ§μ μμ΄ λ§Ήλͺ©μ μΈ μ νλ€κ°νλ κΈ°μ
μ±μ₯μ μ μλ―Έν μν₯μ λ―ΈμΉμ§ μλλ€. μ΄λ κΈ°μ
μ΄ νΉμ λΆμΌμ λν μ§μμ μ§μμ μΌλ‘ μΆμ νκ³ , μΆμ λ μ§μμ λ€μν μμμ μ νκ΅°μ μ μ©νλ μ λ΅μ΄ μ€μν¨μ μμ¬νλ€.
μ°κ΅¬μ κ²°κ³Όλ₯Ό μ’
ν©νλ©΄, κΈ°μ
μ κ²½μμ°μλ₯Ό νλ, μ μ§ν¨μ μμ΄μ νμ νλμ μ, μλ‘μ보λ€λ κΎΈμ€ν νμ΅κ³Ό ν λΆμΌμμμ μ§μμ μΈ κ²½ν, μΆμ μ΄ μ€μνλ€. μ΅κ·Ό νκ΅κΈ°μ
λ€μ μ±κ³Ό λ° κΈ°μ κ²½μλ ₯ μ ν μμΈμ νμ νλμ΄ λΆμ‘±ν΄μκ° μλλΌ νμ νλμ μ§μμ μΌλ‘ νμ§ λͺ»νκΈ° λλ¬Έμ΄λ€. λ³Έ μ°κ΅¬μμ νμ νλμ μ΄ν΄νλ μλ‘μ΄ μΆμΌλ‘ μ§μμ±μ μ μν¨μΌλ‘μ¨ νμ κ³Ό κΈ°μ
μ±μ₯ κ΄κ³λ₯Ό λ³΄λ€ μ²΄κ³μ μΌλ‘ μ΄ν΄ν μ μλ€. λμκ°μ μλ‘μ΄ κ΄μ μ νμ μ λ΅ λ° μ μ±
ν¨μλ₯Ό λμΆν μ μλ€. μ μ°μ
λ°κ΅΄, μ κΈ°μ κ°λ° λλ νμ νλμ μ¦κ°μ λν λ§Ήμ 보λ€λ νμ νλμ μ§μν μ μλ μ¬λ ₯μ ν보νκ³ νκ²½μ μ‘°μ±ν μ μλλ‘ μ λ΅μ αμ μ±
μ λ
Έλ ₯μ΄ μꡬλλ€.Chapter 1. Introduction 1
1.1 Research motivation and purpose 1
1.2 Outline of the study 8
1.3 Contributions of the study 10
Chapter 2. Literature Review and Theoretical Frameworks 13
2.1 Firm growth and innovation 13
2.2 Persistence of innovation activities 62
2.3 Linking persistence, knowledge accumulation and firm growth 76
Chapter 3. Relationship between R&D activities and firm growth: The level and volatility of R&D investment 78
3.1 Introduction 78
3.2 Literature review and hypotheses development 80
3.3 Methodology 90
3.4 Empirical results 97
3.5 Robustness 107
3.6 Sub-conclusion 112
Chapter 4. Effects of knowledge accumulation strategies through experience and experimentation on firm growth 114
4.1 Introduction 114
4.2 Theoretical background and hypotheses development 116
4.3 Methodology 122
4.4 Stylized facts of Korean manufacturing firms 134
4.5 Empirical results 139
4.6 Additional analysis: Types of experimentation 149
4.7 Sub-conclusion 154
Chapter 5. Dynamic relationship between knowledge and product: Innovation strategy for firm growth 156
5.1 Introduction 156
5.2 Literature review and theoretical framework 158
5.3 Data and descriptive statistics 163
5.4 Dynamic relationship between knowledge and product spaces 167
5.5 Innovation strategy for firm growth 175
5.6 Sub-conclusion 185
Chapter 6. Conclusion 187
6.1 Summary 187
6.2 Implications 189
6.3 Limitations and future research directions 193
Bibliography 196
Appendix 1: Distribution of R&D level (Chapter 3) 223
Appendix 2: Results of selection equation (Chapter 4) 224
Appendix 3: Industrial classification (Chapter 4) 225
Appendix 4: Results of quantile regression (Chapter 4) 226
Appendix 5: Descriptive statistics and correlations (Chapter 5) 227
Abstract (Korean) 229Docto
νκ΅μΈ λ¨μν μν¬μ± ννΌλ°λ¦¬μ¦ νμμμμ μΌλΌν΄ 5λ² λ° μΌλΌν΄ 14λ² λμ°λ³μ΄ λΆμ
Dept. of Medicine/μμ¬[νκΈ]λ¨μν μν¬μ± ννΌλ°λ¦¬μ¦(Epidermolysis bullosa simplex, μ΄ν EBS)μ ννΌ μΈν¬λ΄μ μΌλΌν΄ 5λ² λλ 14λ² μ μ μμ λμ°λ³μ΄μ μν΄ λ°μνλ μ μ μ± μν¬μ± μ§νμΌλ‘ λκ° μμΌμ체 μ°μ±ννλ‘ μ μ λλ€. λ³Έ μ§νμ μΈν¬ 골격μ μ μ§νλλ° μ€μν λ¨λ°±μ§μΈ μΌλΌν΄μ νμ± μ΄μμΌλ‘ κ°λ²Όμ΄ μΈλΆ μκ·Ήμλ μ½κ² μν¬κ° μμ±λλ©° μ‘°μ§ μ견μ νΉμ§μ μΌλ‘ κΈ°μ μΈν¬μ μΈν¬μ§ λ΄μμ μΈν¬μ λΆλ¦¬κ° λνλλ€. νμλ μμμ μ€λ±λμ λ°λΌ μ¬ν λ³λ³ μμμ λλ Dowling-Mearaν(EBS-DM), μ μ μ μΈ μμμ 보μ΄λ Koebnerν(EBS-K), κ·Έλ¦¬κ³ κ΅νμ± λ³λ³ λΆν¬μ κ²½ν μμμ λνλ΄λ Weber-Cockayneν(EBS-WC)μΌλ‘ λΆλ₯νλ©° μ΄μ μ μ°κ΅¬μ μνλ©΄ μ΄λ¬ν μμμ μΈ μ€λ±λμ μΌλΌν΄ λ¨λ°± μ μ μμ μ΄μ λΆμμ λ°μ ν μκ΄ κ΄κ³κ° μμμ΄ μλ €μ§κ³ μλ€. Dowling-Mearaνμ κ²½μ° μΌλΌν΄ λ¨λ°±μ λ§λ¨ λΆμμ μ‘΄μ¬νλ λ§€μ° λ³΄μ‘΄λ λ§λ λλ©μΈμ μμΉμμ λλΆλΆ λ³μ΄κ° λ°κ²¬λλ κ²μΌλ‘ λ³΄κ³ λκ³ μμΌλ©°, K?bnerνκ³Ό Weber-Cockayneνμ κ²½μ°λ μκΈ° λΆμ μ΄μΈμ μμΉμμ λ€μ λ°κ²¬λλ κ²μΌλ‘ μλ €μ Έ μλ€. νμ¬κΉμ§ 130μ¬ κ°μ μΌλΌν΄ 5λ² λ° 14λ² μ μ μμ μ΄μμ΄ μλ €μ Έ μμΌλ κ΅λ΄μ EBSμ λν μ°κ΅¬λ μμ§ μ λλ‘ μ΄λ£¨μ΄μ§μ§ μκ³ μλ μνμ΄λ€.
λ³Έ μ°κ΅¬λ 16λͺ
μ νκ΅μΈ EBSνμ λ° κ·Έ κ°κ³λ₯Ό λμμΌλ‘ νμκ³ , λμ° λ³μ΄ λΆμ κ²°κ³Ό λͺ¨λ νμμμ λμ°λ³μ΄κ° λ°κ²¬λμλ€. μ΄ 16건μ 13κ°μ missene, 2κ°μ nonsense, 1 κ°μ small deletion λμ°λ³μ΄λ‘ λ°νμ‘λ€. μ΄μ€ κΈ°μ‘΄μ λ³΄κ³ λμ§ μμ 7건μ μλ‘μ΄ λμ°λ³μ΄λ₯Ό λ°κ²¬νμμΌλ©°, μΌλΌν΄ 5λ²μμ 4κ°(p.V143F, p.R264P, p.C479X and p.Asn177del), μΌλΌν΄ 14λ²μμ 3κ°(p.R125L, p.E392X and p.L401P)κ° κ°κ° λ°κ²¬λμλ€. Dowling-MearaνμΌλ‘ μ§λ¨λ 5λͺ
(K14-p.R125L, K-14p.R125H, K5-p.C479X, K5-p.E477K, K5-p.Asn177del)μ λΆμ κ²°κ³Ό λμ°λ³μ΄μ μμΉκ° λͺ¨λ μΌλΌν΄ λ¨λ°±μ§μ λ§€μ° λ³΄μ‘΄λ λ§λ λλ©μΈμ λ§λ¨ λΆμμ μμΉν¨μ νμΈνμλ€. K?bnerνκ³Ό Weber-CockayneνμΌλ‘ μ§λ¨λ νμμ λμ°λ³μ΄λ μΌλΆ μμΈκ° μμΌλ μ£Όλ‘ μκΈ° λΆμ μ΄μΈμ μμΉμμ λ°κ²¬λμλ€. λν ν νμμ κ²½μ°(homozygous c.1174G>T, K14-p.E392X/p.E392X)λ λλ¬Έ κ²μΌλ‘ μλ €μ§ μμΌμ체 μ΄μ± ννμ EBSλ‘ νμΈλμλ€. μ΄μμ μ°κ΅¬ κ²°κ³Όλ‘ κ΅λ΄μ EBSνμμμ λΉκ΅μ νΉμ§μ μΈ μ μ νκ³Ό νννμ μκ΄κ΄κ³κ° λνλ¨μ νμΈνμκ³ μ΄λ EBSμ λν μ°λ¦¬μ μ΄ν΄λ₯Ό λνκ³ , μΆν μ°μ μ§λ¨ λ° μν μμΈ‘, μ μ μ μΉλ£μ κ°λ°μ λμμ΄ λλ μλ£λ‘ μ΄μ©λ μ μμ κ²μΌλ‘ μ¬κ²¨μ§λ€.
[μλ¬Έ]Epidermolysis bullosa simplex (EBS) is a group of hereditary bullous disorders caused by mutations of the keratin genes KRT5 and KRT14. Patients present with widely varying severity and are classified into three main subtypes, EBS Weber-Cockayne (EBS-WC), EBS Koebner (EBS-K), and EBS Dowling-Meara (EBS-DM), on the basis of the distribution and severity of blisters. Detailed knowledge of the spectrum of EBS mutations and their genotype-phenotype correlation is essential for accurate genetic counseling, prenatal diagnosis and prediction of prognosis. To date, about 130 mutations associated with EBS have been identified in KRT5 and KRT14. A significant correlation between the position of the mutations within these proteins and the clinical severity of EBS has been noted.
In this study, we investigated 16 Korean EBS patients and their families by performing a sequence analysis of the entire coding sequences of KRT5 and KRT14. Pathogenic mutations were found in all cases. We have identified seven novel mutations: four mutations on the KRT5 (p.V143F, p.R265P, p.C479X and p.Asn177del), and three on the KRT14 (p.R125L, p.E392X and p.L401P). Nine mutations that have previously been reported were found within the KRT5 and KRT14 genes. Specifically, thirteen missense, two nonsense, and one small deletion mutation were found. Five of sixteen EBS patients were diagnosed with EBS-DM, and all mutations (K14-p.R125H, K14-p.R125L, K5-E477K, K5-p.C479X, K5-p.Asn177del) were located on the highly conserved ends of the alpha-helical rod domain, the helix initiation (HIP), or helix termination (HTP) peptides of KRT5 and KRT14. Eight and three mutations were reported in EBS-K and EBS-WC, respectively. The positions of mutation in both subtypes are more widely distributed within the rod domains and in the L12 linker domains of both keratin genes. Interestingly, K14-p.E392X/p.E392X had a homozygous recessive profile (c.1174G>T), and the patient showed a EBS-K phenotype. This study shows possible implications of the novel mutations on protein structure, keratin intermediate filament (KIF) formation and the corresponding phenotypes.ope
Measurement of the complex permittivity of dielectrics using cylindrical cavity resonators
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Extrapolation and Correlation in Radiated Emission Measurements and Uncertainty in EMC Measurements
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A study of the triangular cross section type greenhouse using fluid-structure interaction
The purpose of this study is to study the fluid-dynamic and structural characteristics of the conventional greenhouse and to find possible improvement on the current greenhouse. The greenhouse is required to have enough rigidity of the structure while the installation and reinforcement should be as easy as possible. In this study, the structural stability to the snow load was tested through the computational structure analysis based on the building structure standard, and the wind load was computed by computational fluid-structure interaction analysis. The current analysis can be used as a reference data for a new greenhouse and it will be economically viable by reducing installation and maintenance costs.22Nkciothe