6 research outputs found
'Red flags of corruption'in world bank projects : an analysis of infrastructure contracts
"Red flags"are indicators of potential issues regarding governance failure, collusion or corruption in projects. While some specific red flags can be powerful indicators of issues to be addressed, the hypothesis of this paper is that many proposed red flags are potentially too ubiquitous and randomly distributed to be useful as indicators of significant governance failure. The paper examines project documentation from a small sample of World Bank water and sanitation projects in an attempt to collect data on the presence or absence of 13 commonly accepted red flags. This paper finds that: (i) almost every contract reviewed raised at least one of 13 red flags analyzed; (ii) potentially tainted contracts did not exhibit notably more red flags than control contracts; and (iii) the occurrence of multiple red flags in the same contract was rare enough to suggest that joint occurrence was largely by chance, not as a result of a strongly causal inter-relationship between flags. The ubiquity and apparent randomness of these red flags suggests that their roll-out as a monitoring tool requires additional thought as to interpretation, context and use. The paper examines an alternate tool for uncovering potential problem projects -- supplier concentration. Across a very small sample, there does appear to be a relationship between such concentration and potential problem projects.Post Conflict Reconstruction,Debt Markets,Government Procurement,Investment and Investment Climate,Literature&Folklore
Intensity of Russian Companiesβ Mergers & Acquisitions (M&A) Processes, 2001-2004: Econometric Estimation
The article uses econometric tools to analyze and identify main characteristics of mergers & acquisitions of Russian companies at the time of economic growth. We employ the Poisson regression to model M&A behavior, and its relation with macroeconomic indicators, while controlling for a linear trend and seasonality. It is one of the first attempts to estimate a similar model for Russian M&A.mergers and acquisitions; Poisson regression
Investing in Russia: Current Portfolio of Private Equity Funds and Impact of Global Financial Crisis
Π£ΡΠΈΠ»Π΅Π½ΠΈΠ΅ ΡΠΊΠΎΠ½ΠΎΠΌΠΈΡΠ΅ΡΠΊΠΈΡ ΠΏΠΎΠ·ΠΈΡΠΈΠΉ ΡΠΎΡΡΠΈΠΉΡΠΊΠΎΠΉ ΡΠ»Π΅ΠΊΡΡΠΎΠ½ΠΈΠΊΠΈ: ΠΌΠ΅Ρ Π°Π½ΠΈΠ·ΠΌΡ ΠΈ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎΡΡΠΈ
The research analyzes the changes in economic relations between the state and economic entities in the electronics market. It is for the first time when we discuss the authorβs classification, which identifies four types of models of interaction between the state and Russian electronics companies depending on changes in the business cycle and instability of the economy: an inert or passive observer, a crisis model, dynamic and rational participation, and a model of active and multi-agency interactions. Specific characteristics of the proposed models are identified, and the features of interaction between government structures and companies are systematized. The research shows an increasing role of the state in supporting and financing new directions of the development of integrated structures in electronics, despite the processes of globalization and liberalization of the national economy. The novelty of the research is in the development of a comprehensive view on the institutional structure of state support for electronics. An active process of formation of complex-structured economic entities at the industry, inter-industry and interregional levels on the basis of state strategies for the development of electronics is analyzed. The specificity of modern behaviour of Russian electronics companies in the digital economy and the coronavirus pandemic is revealed on the basis of generalization of stylized facts of the specific companiesβ activitiesΠΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅ ΠΏΠΎΡΠ²ΡΡΠ΅Π½ΠΎ Π°Π½Π°Π»ΠΈΠ·Ρ ΠΈΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΠΉ Ρ
ΠΎΠ·ΡΠΉΡΡΠ²Π΅Π½Π½ΡΡ
ΠΎΡΠ½ΠΎΡΠ΅Π½ΠΈΠΉ ΠΌΠ΅ΠΆΠ΄Ρ Π³ΠΎΡΡΠ΄Π°ΡΡΡΠ²ΠΎΠΌ ΠΈ ΡΡΠ±ΡΠ΅ΠΊΡΠ°ΠΌΠΈ ΡΠΊΠΎΠ½ΠΎΠΌΠΈΠΊΠΈ Π½Π° ΡΡΠ½ΠΊΠ΅ ΡΠ»Π΅ΠΊΡΡΠΎΠ½ΠΈΠΊΠΈ. ΠΠΏΠ΅ΡΠ²ΡΠ΅
ΠΎΠ±ΡΡΠΆΠ΄Π°Π΅ΡΡΡ Π°Π²ΡΠΎΡΡΠΊΠ°Ρ ΠΊΠ»Π°ΡΡΠΈΡΠΈΠΊΠ°ΡΠΈΡ, Π²ΡΠ΄Π΅Π»ΡΡΡΠ°Ρ ΡΠ΅ΡΡΡΠ΅ ΡΠΈΠΏΠ° ΠΌΠΎΠ΄Π΅Π»Π΅ΠΉ Π²Π·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΠΈΡ Π³ΠΎΡΡΠ΄Π°ΡΡΡΠ²Π° ΠΈ ΡΠΎΡΡΠΈΠΉΡΠΊΠΈΡ
ΠΊΠΎΠΌΠΏΠ°Π½ΠΈΠΉ ΡΠ»Π΅ΠΊΡΡΠΎΠ½ΠΈΠΊΠΈ Π² Π·Π°Π²ΠΈΡΠΈΠΌΠΎΡΡΠΈ ΠΎΡ ΠΈΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΡ Π΄Π΅Π»ΠΎΠ²ΠΎΠ³ΠΎ ΡΠΈΠΊΠ»Π° ΠΈ Π½Π΅ΡΡΠ°Π±ΠΈΠ»ΡΠ½ΠΎΡΡΠΈ ΡΠΊΠΎΠ½ΠΎΠΌΠΈΠΊΠΈ: ΠΈΠ½Π΅ΡΡΠ½ΠΎΠ³ΠΎ ΠΈΠ»ΠΈ ΠΏΠ°ΡΡΠΈΠ²Π½ΠΎΠ³ΠΎ
Π½Π°Π±Π»ΡΠ΄Π°ΡΠ΅Π»Ρ, ΠΊΡΠΈΠ·ΠΈΡΠ½Π°Ρ, Π΄ΠΈΠ½Π°ΠΌΠΈΡΠ½ΠΎΠ³ΠΎ ΠΈ ΡΠ°ΡΠΈΠΎΠ½Π°Π»ΡΠ½ΠΎΠ³ΠΎ ΡΡΠ°ΡΡΠΈΡ, ΠΌΠΎΠ΄Π΅Π»Ρ Π°ΠΊΡΠΈΠ²Π½ΡΡ
ΠΈ ΠΌΡΠ»ΡΡΠΈΠ°Π³Π΅Π½ΡΡΠΊΠΈΡ
Π²Π·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΠΈΠΉ. ΠΡΡΠ²Π»Π΅Π½Ρ ΡΠΏΠ΅ΡΠΈΡΠΈΡΠ΅ΡΠΊΠΈΠ΅ Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΡΡΠΈΠΊΠΈ
ΠΏΡΠ΅Π΄Π»ΠΎΠΆΠ΅Π½Π½ΡΡ
ΠΌΠΎΠ΄Π΅Π»Π΅ΠΉ, ΡΠΈΡΡΠ΅ΠΌΠ°ΡΠΈΠ·ΠΈΡΠΎΠ²Π°Π½Ρ ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ Π²Π·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΠΈΡ Π²Π»Π°ΡΡΠ½ΡΡ
ΡΡΡΡΠΊΡΡΡ ΠΈ ΠΊΠΎΠΌΠΏΠ°Π½ΠΈΠΉ. ΠΠΎΠΊΠ°Π·Π°Π½ΠΎ Π²ΠΎΠ·ΡΠ°ΡΡΠ°Π½ΠΈΠ΅ ΡΠΎΠ»ΠΈ Π³ΠΎΡΡΠ΄Π°ΡΡΡΠ²Π° Π² ΠΏΠΎΠ΄Π΄Π΅ΡΠΆΠΊΠ΅
ΠΈ ΡΠΈΠ½Π°Π½ΡΠΈΡΠΎΠ²Π°Π½ΠΈΠΈ Π½ΠΎΠ²ΡΡ
Π½Π°ΠΏΡΠ°Π²Π»Π΅Π½ΠΈΠΉ ΡΠ°Π·Π²ΠΈΡΠΈΡ ΠΈΠ½ΡΠ΅Π³ΡΠΈΡΠΎΠ²Π°Π½Π½ΡΡ
ΡΡΡΡΠΊΡΡΡ Π² ΡΠ»Π΅ΠΊΡΡΠΎΠ½ΠΈΠΊΠ΅, Π½Π΅ΡΠΌΠΎΡΡΡ Π½Π° ΠΏΡΠΎΡΠ΅ΡΡΡ Π³Π»ΠΎΠ±Π°Π»ΠΈΠ·Π°ΡΠΈΠΈ ΠΈ Π»ΠΈΠ±Π΅ΡΠ°Π»ΠΈΠ·Π°ΡΠΈΠΈ Π½Π°ΡΠΈΠΎΠ½Π°Π»ΡΠ½ΠΎΠΉ
ΡΠΊΠΎΠ½ΠΎΠΌΠΈΠΊΠΈ. ΠΠΎΠ²ΠΈΠ·Π½Π° ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΡ Π·Π°ΠΊΠ»ΡΡΠ°Π΅ΡΡΡ Π² ΡΠ°Π·ΡΠ°Π±ΠΎΡΠΊΠ΅ ΠΊΠΎΠΌΠΏΠ»Π΅ΠΊΡΠ½ΠΎΠ³ΠΎ ΠΏΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½ΠΈΡ ΠΎΠ± ΠΈΠ½ΡΡΠΈΡΡΡΠΈΠΎΠ½Π°Π»ΡΠ½ΠΎΠΉ ΡΡΡΡΠΊΡΡΡΠ΅ Π³ΠΎΡΡΠ΄Π°ΡΡΡΠ²Π΅Π½Π½ΠΎΠΉ ΠΏΠΎΠ΄Π΄Π΅ΡΠΆΠΊΠΈ ΡΠ»Π΅ΠΊΡΡΠΎΠ½ΠΈΠΊΠΈ. ΠΠ½Π°Π»ΠΈΠ·ΠΈΡΡΠ΅ΡΡΡ Π°ΠΊΡΠΈΠ²Π½ΡΠΉ ΠΏΡΠΎΡΠ΅ΡΡ ΡΠΎΡΠΌΠΈΡΠΎΠ²Π°Π½ΠΈΡ ΡΠ»ΠΎΠΆΠ½ΠΎΡΡΡΡΠΊΡΡΡΠΈΡΠΎΠ²Π°Π½Π½ΡΡ
Ρ
ΠΎΠ·ΡΠΉΡΡΠ²ΡΡΡΠΈΡ
ΡΡΠ±ΡΠ΅ΠΊΡΠΎΠ² ΠΎΡΡΠ°ΡΠ»Π΅Π²ΠΎΠ³ΠΎ, ΠΌΠ΅ΠΆΠΎΡΡΠ°ΡΠ»Π΅Π²ΠΎΠ³ΠΎ ΠΈ ΠΌΠ΅ΠΆΡΠ΅Π³ΠΈΠΎΠ½Π°Π»ΡΠ½ΠΎΠ³ΠΎ
ΡΡΠΎΠ²Π½Π΅ΠΉ Π½Π° Π±Π°Π·Π΅ Π³ΠΎΡΡΠ΄Π°ΡΡΡΠ²Π΅Π½Π½ΡΡ
ΡΡΡΠ°ΡΠ΅Π³ΠΈΠΉ ΡΠ°Π·Π²ΠΈΡΠΈΡ ΡΠ»Π΅ΠΊΡΡΠΎΠ½ΠΈΠΊΠΈ. ΠΠ±ΡΡΠΆΠ΄Π°Π΅ΡΡΡ
ΡΠΎΠ²ΡΠ΅ΠΌΠ΅Π½Π½ΠΎΠ΅ ΠΏΠΎΠ²Π΅Π΄Π΅Π½ΠΈΠ΅ ΡΠΎΡΡΠΈΠΉΡΠΊΠΈΡ
ΠΊΠΎΠΌΠΏΠ°Π½ΠΈΠΉ ΡΠ»Π΅ΠΊΡΡΠΎΠ½ΠΈΠΊΠΈ Π² ΡΡΠ»ΠΎΠ²ΠΈΡΡ
ΡΠΈΡΡΠΎΠ²ΠΎΠΉ
ΡΠΊΠΎΠ½ΠΎΠΌΠΈΠΊΠΈ ΠΈ ΠΏΠ°Π½Π΄Π΅ΠΌΠΈΠΈ ΠΊΠΎΡΠΎΠ½Π°Π²ΠΈΡΡΡΠ° Π½Π° ΠΎΡΠ½ΠΎΠ²Π΅ ΠΎΠ±ΠΎΠ±ΡΠ΅Π½ΠΈΡ ΡΡΠΈΠ»ΠΈΠ·ΠΎΠ²Π°Π½Π½ΡΡ
ΡΠ°ΠΊΡΠΎΠ²
Π΄Π΅ΡΡΠ΅Π»ΡΠ½ΠΎΡΡΠΈ ΠΊΠΎΠ½ΠΊΡΠ΅ΡΠ½ΡΡ
ΠΊΠΎΠΌΠΏΠ°Π½ΠΈ
Π€ΠΎΡΠΌΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅ ΠΏΡΠΎΠ°ΠΊΡΠΈΠ²Π½ΡΡ ΡΡΡΠ°ΡΠ΅Π³ΠΈΠΉ ΠΊΠΈΡΠ°ΠΉΡΠΊΠΈΡ ΡΠΎΠ½Π΄ΠΎΠ² ΠΏΡΡΠΌΡΡ ΡΠ°ΡΡΠ½ΡΡ ΠΈΠ½Π²Π΅ΡΡΠΈΡΠΈΠΉ Π½Π° ΡΠΎΡΡΠΈΠΉΡΠΊΠΎΠΌ ΡΡΠ½ΠΊΠ΅ ΠΊΠΎΡΠΏΠΎΡΠ°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ ΠΊΠΎΠ½ΡΡΠΎΠ»Ρ
The article discusses the boundaries and contours of the possible institutional behavior of private equity funds (PEFs) in the Russian Federation in modern conditions. It analyzes the transformation of PEF sectoral interests in the Russian assets of non-public companies in the period of economic instability. Besides, it gives a relevant assessment of changes in the regional aspects of interaction between PEFs and recipient companies from Russia against the background of sanctions and import substitution, as well as private equity cycle. The article presents the modern metrics of Chinese PEFs and a multi-level monitoring of existing Chinese PEF projects in the Russian economy. It also analyzes the effects of multi-agent relations of PEFs and target companies in Russia against the background of a gradual recovery of the countryβs economic growth. The article discusses the current and preferred format for the participation of investors in the PE market in the context of the adaptation of the Russian economy to the sanctions regime. It identifies the factors affecting the prospects and dynamics of the development of Chinese PEFs with an investment mandate for Russia and discusses the mechanisms of institutional support for expanding the presence of Chinese PEs in the Russian market for corporate controlΠ ΡΡΠ°ΡΡΠ΅ ΡΠ°ΡΡΠΌΠΎΡΡΠ΅Π½Ρ Π³ΡΠ°Π½ΠΈΡΡ ΠΈ ΠΊΠΎΠ½ΡΡΡΡ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎΠ³ΠΎ ΠΈΠ½ΡΡΠΈΡΡΡΠΈΠΎΠ½Π°Π»ΡΠ½ΠΎΠ³ΠΎ ΠΏΠΎΠ²Π΅Π΄Π΅Π½ΠΈΡ ΡΠΎΠ½Π΄ΠΎΠ² ΠΏΡΡΠΌΡΡ
ΡΠ°ΡΡΠ½ΡΡ
ΠΈΠ½Π²Π΅ΡΡΠΈΡΠΈΠΉ (PEF) Π² Π Π€ Π² ΡΠΎΠ²ΡΠ΅ΠΌΠ΅Π½Π½ΡΡ
ΡΡΠ»ΠΎΠ²ΠΈΡΡ
. ΠΠ½Π°Π»ΠΈΠ·ΠΈΡΡΠ΅ΡΡΡ ΡΡΠ°Π½ΡΡΠΎΡΠΌΠ°ΡΠΈΡ ΠΎΡΡΠ°ΡΠ»Π΅Π²ΡΡ
ΠΈΠ½ΡΠ΅ΡΠ΅ΡΠΎΠ² ΡΠΎ ΡΡΠΎΡΠΎΠ½Ρ PEF ΠΊ ΡΠΎΡΡΠΈΠΉΡΠΊΠΈΠΌ
Π°ΠΊΡΠΈΠ²Π°ΠΌ Π½Π΅ΠΏΡΠ±Π»ΠΈΡΠ½ΡΡ
ΠΊΠΎΠΌΠΏΠ°Π½ΠΈΠΉ Π² ΠΏΠ΅ΡΠΈΠΎΠ΄ ΡΠΊΠΎΠ½ΠΎΠΌΠΈΡΠ΅ΡΠΊΠΎΠΉ Π½Π΅ΡΡΠ°Π±ΠΈΠ»ΡΠ½ΠΎΡΡΠΈ. ΠΠ°Π½Π° ΡΠ΅Π»Π΅Π²Π°Π½ΡΠ½Π°Ρ ΠΎΡΠ΅Π½ΠΊΠ° ΠΈΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΡ ΡΠ΅Π³ΠΈΠΎΠ½Π°Π»ΡΠ½ΡΡ
Π°ΡΠΏΠ΅ΠΊΡΠΎΠ² Π²Π·Π°ΠΈΠΌΠΎΠ΄Π΅ΠΉΡΡΠ²ΠΈΡ PEF ΠΈ ΠΊΠΎΠΌΠΏΠ°Π½ΠΈΠΉ-ΡΠ΅ΡΠΈΠΏΠΈΠ΅Π½ΡΠΎΠ²
ΠΈΠ· Π ΠΎΡΡΠΈΠΈ Π½Π° ΡΠΎΠ½Π΅ ΡΠ°Π½ΠΊΡΠΈΠΉ ΠΈ ΠΈΠΌΠΏΠΎΡΡΠΎΠ·Π°ΠΌΠ΅ΡΠ΅Π½ΠΈΡ, ΡΠΈΠΊΠ»ΠΈΡΠ½ΠΎΡΡΠΈ ΡΡΠ½ΠΊΠ°
(PE). ΠΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Ρ ΡΠΎΠ²ΡΠ΅ΠΌΠ΅Π½Π½ΡΠ΅ ΠΌΠ΅ΡΡΠΈΠΊΠΈ ΠΊΠΈΡΠ°ΠΉΡΠΊΠΈΡ
PEF, ΠΎΡΡΡΠ΅ΡΡΠ²Π»Π΅Π½ ΠΌΠ½ΠΎΠ³ΠΎΡΡΠΎΠ²Π½Π΅Π²ΡΠΉ ΠΌΠΎΠ½ΠΈΡΠΎΡΠΈΠ½Π³ Π΄Π΅ΠΉΡΡΠ²ΡΡΡΠΈΡ
ΠΏΡΠΎΠ΅ΠΊΡΠΎΠ² ΠΊΠΈΡΠ°ΠΉΡΠΊΠΈΡ
PΠF Π² ΡΠΊΠΎΠ½ΠΎΠΌΠΈΠΊΠ΅ Π ΠΎΡΡΠΈΠΈ. ΠΡΠΎΠ²Π΅Π΄Π΅Π½
Π°Π½Π°Π»ΠΈΠ· ΠΏΠΎΡΠ»Π΅Π΄ΡΡΠ²ΠΈΠΉ ΠΌΡΠ»ΡΡΠΈΠ°Π³Π΅Π½ΡΡΠΊΠΈΡ
ΠΎΡΠ½ΠΎΡΠ΅Π½ΠΈΠΉ PEF ΠΈ ΡΠ΅Π»Π΅Π²ΡΡ
ΠΊΠΎΠΌΠΏΠ°Π½ΠΈΠΉ Π² Π ΠΎΡΡΠΈΠΈ
Π½Π° ΡΠΎΠ½Π΅ ΠΏΠΎΡΡΠ΅ΠΏΠ΅Π½Π½ΠΎΠ³ΠΎ Π²ΠΎΡΡΡΠ°Π½ΠΎΠ²Π»Π΅Π½ΠΈΡ ΡΠΊΠΎΠ½ΠΎΠΌΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΡΠΎΡΡΠ° ΡΡΡΠ°Π½Ρ. ΠΠ±ΡΡΠΆΠ΄Π°Π΅ΡΡΡ ΡΠ»ΠΎΠΆΠΈΠ²ΡΠΈΠΉΡΡ ΠΈ ΠΏΡΠ΅Π΄ΠΏΠΎΡΠΈΡΠ°Π΅ΠΌΡΠΉ ΡΠΎΡΠΌΠ°Ρ ΡΡΠ°ΡΡΠΈΡ ΠΈΠ½Π²Π΅ΡΡΠΎΡΠΎΠ² Π½Π° ΡΡΠ½ΠΊΠ΅ PE Π² ΡΡΠ»ΠΎΠ²ΠΈΡΡ
Π°Π΄Π°ΠΏΡΠ°ΡΠΈΠΈ ΡΠΎΡΡΠΈΠΉΡΠΊΠΎΠΉ ΡΠΊΠΎΠ½ΠΎΠΌΠΈΠΊΠΈ ΠΊ ΡΠ°Π½ΠΊΡΠΈΠΎΠ½Π½ΠΎΠΌΡ ΡΠ΅ΠΆΠΈΠΌΡ. ΠΡΡΠ²Π»Π΅Π½Ρ ΡΠ°ΠΊΡΠΎΡΡ,
Π²Π»ΠΈΡΡΡΠΈΠ΅ Π½Π° ΠΏΠ΅ΡΡΠΏΠ΅ΠΊΡΠΈΠ²Ρ ΠΈ Π΄ΠΈΠ½Π°ΠΌΠΈΠΊΡ ΡΠ°Π·Π²ΠΈΡΠΈΡ ΠΊΠΈΡΠ°ΠΉΡΠΊΠΈΡ
PEF Ρ ΠΈΠ½Π²Π΅ΡΡΠΈΡΠΈΠΎΠ½Π½ΡΠΌ
ΠΌΠ°Π½Π΄Π°ΡΠΎΠΌ Π½Π° Π ΠΎΡΡΠΈΡ. ΠΠ±ΡΡΠΆΠ΄Π°ΡΡΡΡ ΠΌΠ΅Ρ
Π°Π½ΠΈΠ·ΠΌΡ ΠΈΠ½ΡΡΠΈΡΡΡΠΈΠΎΠ½Π°Π»ΡΠ½ΠΎΠΉ ΠΏΠΎΠ΄Π΄Π΅ΡΠΆΠΊΠΈ ΡΠ°ΡΡΠΈΡΠ΅Π½ΠΈΡ ΠΏΡΠΈΡΡΡΡΡΠ²ΠΈΡ ΠΊΠΈΡΠ°ΠΉΡΠΊΠΈΡ
PE Π½Π° ΡΠΎΡΡΠΈΠΉΡΠΊΠΎΠΌ ΡΡΠ½ΠΊΠ΅ ΠΊΠΎΡΠΏΠΎΡΠ°ΡΠΈΠ²Π½ΠΎΠ³ΠΎ ΠΊΠΎΠ½ΡΡΠΎΠ»
Ultrastructural Abnormalities in Induced Pluripotent Stem Cell-Derived Neural Stem Cells and Neurons of Two Cohen Syndrome Patients
Cohen syndrome is an autosomal recessive disorder caused by VPS13B (COH1) gene mutations. This syndrome is significantly underdiagnosed and is characterized by intellectual disability, microcephaly, autistic symptoms, hypotension, myopia, retinal dystrophy, neutropenia, and obesity. VPS13B regulates intracellular membrane transport and supports the Golgi apparatus structure, which is critical for neuron formation. We generated induced pluripotent stem cells from two patients with pronounced manifestations of Cohen syndrome and differentiated them into neural stem cells and neurons. Using transmission electron microscopy, we documented multiple new ultrastructural changes associated with Cohen syndrome in the neuronal cells. We discovered considerable disturbances in the structure of some organelles: Golgi apparatus fragmentation and swelling, endoplasmic reticulum structural reorganization, mitochondrial defects, and the accumulation of large autophagosomes with undigested contents. These abnormalities underline the ultrastructural similarity of Cohen syndrome to many neurodegenerative diseases. The cell models that we developed based on patient-specific induced pluripotent stem cells can serve to uncover not only neurodegenerative processes, but the causes of intellectual disability in general