From PHYS.org, UCD Conway Institute
Insight on molecular regulation of cilium structure and composition
Scanning electron microscope image of lung trachea epithelium. There are both ciliated and non-ciliated cells in this epithelium. Note the difference in size between the cilia and the microvilli (on the non-ciliated cell surface). Credit: Charles Daghlian via Wikimedia Commons
The surfaces of most cell types are covered by hair-like cilia that play critical roles in cell and fluid motility, environment sensing, and cell-cell communication (signalling).
New research from University College Dublin shows that cells use a common set of proteins in different ways to build cilia with distinct structures and functions.
This shared set of proteins is also shown to have distinct roles in restricting particular 'gating' components to the base of cilia that control the molecular composition of the structure.
Defects in cilia cause a wide range of developmental diseases (ciliopathies) that affect nearly every organ system in the human body. Examples include polycystic kidney disease, nephronophthisis, retinitis pigmentosa, Bardet–Biedl syndrome, Joubert syndrome, and Meckel-Gruber syndrome.
Associate Professor Oliver Blacque and Dr. Noemie Scheidel from UCD Conway Institute and UCD School of Biomolecular & Biomedical Science are investigating the molecular pathways underpinning cilium biology and disease.
"In this study, we wanted to know how cells use their common intraflagellar transport (IFT) machinery to build cilia with different shapes and functions. We also wanted to determine if IFT and the 'gating' machinery at the ciliary base interact in some way, given that both regulate cilium formation, composition and signalling output," said Associate Professor Blacque.
The intraflagellar transport system occurs along ciliary microtubules (train tracks), employing molecular motors (engines) and at least two types of cargo adaptor complexes (wagons), known as the IFT-A and IFT-B protein complexes.
Dr. Noemie Scheidel carried out the experiments in this study using the nematode (roundworm) model organism, C. elegans. Using genetic and fluorescent imaging techniques, she was able to establish the requirements of different IFT-A genes in building cilia of distinct structure and function.
Dr. Scheidel also used the same gene 'knockouts' and fluorescent versions of 'gating' components to investigate if IFT-A genes control how these molecules are summoned to the cilium base.
"Our new work has revealed that IFT-A complexes are deployed in distinct ways in different cell types and that IFT complexes are involved in ensuring that 'gating' molecules are correctly restricted to their zone of function at the cilia base.
The next step is to bring this work into human cells to see if the findings are like what we found in nematodes," said Dr. Blacque.
He adds, "Ultimately, we want to understand how human cells regulate cilium formation and composition, and provide new research avenues towards ciliary disease understanding and possible therapy."
From CheckBioTech
Ways In Which Stem Cells Can Be Used In The Treatment Of Polycystic Kidney Disease
Polycystic kidney disease is a disease in which clusters of cyst develop in the kidneys. This makes the kidneys big, and also causes it to lose its function progressively. The kidney cysts are noncancerous, and they occur in different sizes. The cysts are usually filled with fluid, and they can develop in infancy, childhood or adulthood. Polycystic kidney disease is caused by a mutation of some genes, which produces a protein that impairs the tubule development. This disease can exist in two forms, which are autosomal dominant polycystic kidney disease, and autosomal recessive polycystic kidney disease. Physicians diagnose this disease based on the signs and symptoms presented by the patient. Some of the signs and symptoms of the disease include painnew onset flank pain, redness of the urine, enlargement of the kidney when examined, and so on. This disease can also cause complications. Examples of complications that could occur as a result of polycystic kidney disease include frequent cyst infections, impairment of the renal function, and so on. Physicians manage this disease by prescribing medications. Antihypertensive are administered to the patient, such as angiotensin converting enzyme, angiotensin receptor blockers, and so on. Infections associated with the disease are also treated with antibiotics, Kidney transplantation could be done in severe cases.
The surfaces of most cell types are covered by hair-like cilia that play critical roles in cell and fluid motility, environment sensing, and cell-cell communication (signalling).
New research from University College Dublin shows that cells use a common set of proteins in different ways to build cilia with distinct structures and functions.
This shared set of proteins is also shown to have distinct roles in restricting particular 'gating' components to the base of cilia that control the molecular composition of the structure.
Defects in cilia cause a wide range of developmental diseases (ciliopathies) that affect nearly every organ system in the human body. Examples include polycystic kidney disease, nephronophthisis, retinitis pigmentosa, Bardet–Biedl syndrome, Joubert syndrome, and Meckel-Gruber syndrome.
Associate Professor Oliver Blacque and Dr. Noemie Scheidel from UCD Conway Institute and UCD School of Biomolecular & Biomedical Science are investigating the molecular pathways underpinning cilium biology and disease.
"In this study, we wanted to know how cells use their common intraflagellar transport (IFT) machinery to build cilia with different shapes and functions. We also wanted to determine if IFT and the 'gating' machinery at the ciliary base interact in some way, given that both regulate cilium formation, composition and signalling output," said Associate Professor Blacque.
The intraflagellar transport system occurs along ciliary microtubules (train tracks), employing molecular motors (engines) and at least two types of cargo adaptor complexes (wagons), known as the IFT-A and IFT-B protein complexes.
Dr. Noemie Scheidel carried out the experiments in this study using the nematode (roundworm) model organism, C. elegans. Using genetic and fluorescent imaging techniques, she was able to establish the requirements of different IFT-A genes in building cilia of distinct structure and function.
Dr. Scheidel also used the same gene 'knockouts' and fluorescent versions of 'gating' components to investigate if IFT-A genes control how these molecules are summoned to the cilium base.
"Our new work has revealed that IFT-A complexes are deployed in distinct ways in different cell types and that IFT complexes are involved in ensuring that 'gating' molecules are correctly restricted to their zone of function at the cilia base.
The next step is to bring this work into human cells to see if the findings are like what we found in nematodes," said Dr. Blacque.
He adds, "Ultimately, we want to understand how human cells regulate cilium formation and composition, and provide new research avenues towards ciliary disease understanding and possible therapy."
From CheckBioTech
Ways In Which Stem Cells Can Be Used In The Treatment Of Polycystic Kidney Disease
Polycystic kidney disease is a disease in which clusters of cyst develop in the kidneys. This makes the kidneys big, and also causes it to lose its function progressively. The kidney cysts are noncancerous, and they occur in different sizes. The cysts are usually filled with fluid, and they can develop in infancy, childhood or adulthood. Polycystic kidney disease is caused by a mutation of some genes, which produces a protein that impairs the tubule development. This disease can exist in two forms, which are autosomal dominant polycystic kidney disease, and autosomal recessive polycystic kidney disease. Physicians diagnose this disease based on the signs and symptoms presented by the patient. Some of the signs and symptoms of the disease include painnew onset flank pain, redness of the urine, enlargement of the kidney when examined, and so on. This disease can also cause complications. Examples of complications that could occur as a result of polycystic kidney disease include frequent cyst infections, impairment of the renal function, and so on. Physicians manage this disease by prescribing medications. Antihypertensive are administered to the patient, such as angiotensin converting enzyme, angiotensin receptor blockers, and so on. Infections associated with the disease are also treated with antibiotics, Kidney transplantation could be done in severe cases.
Stem cells are also used for the treatment of the disease. Stem cells are unique cells that can proliferate, regenerate, repair and replace damaged or injured cells and tissues of the body. This is what makes the therapy effective in the treatment of this disease. Stem cells extracted from the adipose tissues are usually used for this purpose. They are extracted from the patient and re-transplanted to the patient to induce other cells to repair the damaged part of the kidney.
What Are The Signs And Symptoms Of Polycystic Kidney Disease?
Some of the signs and symptoms of polycystic kidney disease include the following;
Hypertension: Patients affected by polycystic kidney disease might present with an elevated blood pressure.
Patients might present with pains on their back, and also on their side.
Headache
Patients might develop a sense of fullness in their abdomen.
Hematuria: This is a condition in which there is blood in the urine.
Patients might also develop kidney stones.
Infections: Patients might develop an infection in the kidney or in the urinary tract.
The abdomen of patients might also become enlarged due to the increase in the size of the kidney.
What’s the Cause of Polycystic Kidney Disease?
As said earlier, this disease is caused by a defected gene that induces the production of an abnormal protein. This protein generally impairs the development of the kidney tubule. There are two types of this disease, and these are autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease. The cause of the disease is specific to each of them.
Autosomal dominant polycystic kidney disease: This is the most predominant type of polycystic Studies have shown that about one in ten cases of patients on dialysis were initially diagnosed and treated for this disease. This disease is more common in people between the ages of thirty and forty. However, children do get affected by this disease. This disease is autosomal dominant, which implies that only a parent is needed to have this disease, for the children to get affected. A child has a fifty per cent chance of having the disease even if one of the parents has the disease.
Autosomal recessive polycystic kidney disease: This disease is far less common when compared to autosomal recessive polycystic kidney disease. The signs and symptoms don’t usually present early until the child grows into adolescence. This disease is generally more predominant in children. It’s an autosomal recessive disease, so both parents must have the abnormal genes for the child to have a chance of having the disease.
What Are The Complications Of Polycystic Kidney Disease?
Below are some of the complications of the disease;
Hypertension: An abnormal increase in blood pressure, is one of the commonest symptoms of polycystic kidney disease. This can lead to other serious complications, especially, when left untreated. Other diseases that might develop as a result of this include renal damage, and an increase in the risk of cardiovascular diseases.
Pregnancy complications: Pregnant patients, affected by polycystic kidney disease usually do not have problems. However, there are some conditions in which they can develop preeclampsia. Women that are hypertensive before getting pregnant, have a high risk of developing this condition.
Colon problems: Patients affected by polycystic kidney disease might develop pouches in their colonic wall. This condition is known as diverticulosis.
Aneurysm in the brain: an Aneurysm is a condition in which there is a wall of the blood vessel develops a balloon-like bulge. This would lead to bleeding if it ruptures. People affected with polycystic kidney disease have a high risk of developing this disease.
Impairment of the renal system: The gradual loss of the functions of the kidney is one of the most prominent symptoms of this disease. According to statistics, more than half of the people affected with this disease usually presents with renal failure by the time they attain the age of sixty. Some of the symptoms of an impaired kidney include the inability of the kidney to eliminate toxic materials from the body.
Preeclampsia: Polycystic kidney disease increases the risk of which pregnant women can develop preeclampsia. This is a condition in which pregnant women experience proteinuria and hypertension during pregnancy.
What Are The Signs And Symptoms Of Polycystic Kidney Disease?
Some of the signs and symptoms of polycystic kidney disease include the following;
Hypertension: Patients affected by polycystic kidney disease might present with an elevated blood pressure.
Patients might present with pains on their back, and also on their side.
Headache
Patients might develop a sense of fullness in their abdomen.
Hematuria: This is a condition in which there is blood in the urine.
Patients might also develop kidney stones.
Infections: Patients might develop an infection in the kidney or in the urinary tract.
The abdomen of patients might also become enlarged due to the increase in the size of the kidney.
What’s the Cause of Polycystic Kidney Disease?
As said earlier, this disease is caused by a defected gene that induces the production of an abnormal protein. This protein generally impairs the development of the kidney tubule. There are two types of this disease, and these are autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease. The cause of the disease is specific to each of them.
Autosomal dominant polycystic kidney disease: This is the most predominant type of polycystic Studies have shown that about one in ten cases of patients on dialysis were initially diagnosed and treated for this disease. This disease is more common in people between the ages of thirty and forty. However, children do get affected by this disease. This disease is autosomal dominant, which implies that only a parent is needed to have this disease, for the children to get affected. A child has a fifty per cent chance of having the disease even if one of the parents has the disease.
Autosomal recessive polycystic kidney disease: This disease is far less common when compared to autosomal recessive polycystic kidney disease. The signs and symptoms don’t usually present early until the child grows into adolescence. This disease is generally more predominant in children. It’s an autosomal recessive disease, so both parents must have the abnormal genes for the child to have a chance of having the disease.
What Are The Complications Of Polycystic Kidney Disease?
Below are some of the complications of the disease;
Hypertension: An abnormal increase in blood pressure, is one of the commonest symptoms of polycystic kidney disease. This can lead to other serious complications, especially, when left untreated. Other diseases that might develop as a result of this include renal damage, and an increase in the risk of cardiovascular diseases.
Pregnancy complications: Pregnant patients, affected by polycystic kidney disease usually do not have problems. However, there are some conditions in which they can develop preeclampsia. Women that are hypertensive before getting pregnant, have a high risk of developing this condition.
Colon problems: Patients affected by polycystic kidney disease might develop pouches in their colonic wall. This condition is known as diverticulosis.
Aneurysm in the brain: an Aneurysm is a condition in which there is a wall of the blood vessel develops a balloon-like bulge. This would lead to bleeding if it ruptures. People affected with polycystic kidney disease have a high risk of developing this disease.
Impairment of the renal system: The gradual loss of the functions of the kidney is one of the most prominent symptoms of this disease. According to statistics, more than half of the people affected with this disease usually presents with renal failure by the time they attain the age of sixty. Some of the symptoms of an impaired kidney include the inability of the kidney to eliminate toxic materials from the body.
Preeclampsia: Polycystic kidney disease increases the risk of which pregnant women can develop preeclampsia. This is a condition in which pregnant women experience proteinuria and hypertension during pregnancy.
This is to inform the general public that Kidney donors are needed from all blood groups by Manipal Hospital, each donor gets ($550,000.00USD). Advance payment will be paid first to the donor before the operation will commence after which the balance will be paid after the completion of the operation. hurry now and contact us Via Email:(manipalhospitalofficial@gmail.com), WHATSAPP NUMBER : +18573099679
ReplyDeleteBest regard : Dr Aritra Ghosh.