The signs and symptoms of mucopolysaccharidosis type I (MPS I) are not present at birth, but. Seven distinct forms and numerous subtypes of mucopolysaccharidosis have been identified. Associated signs and symptoms and the severity of the condition vary significantly by form. In general, most affected people appear healthy at birth and experience a period of normal development, followed by a decline in physical and/or mental function
. Mucopolysaccharidosis (MPS): Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis Sanfilippo syndrome (mucopolysaccharidosis type III; MPS III) has four subtypes (A, B, C, and D) that are distinguished by four different enzyme deficiencies. Initial symptoms of the four types of Sanfilippo syndrome include hyperactivity, sleep disorders, and delays in attaining developmental milestones (e.g., crawling and walking)
Other symptoms include short stature, stunted growth, hernias and respiratory issues. MPS type IX: MPS type IX is caused by the missing or insufficient enzyme, hyaluronidase. As of 2001, there was. Mucopolysaccharidosis. Mucopolysaccharidosis (MPS) is a group of disorders in which a deficiency of certain lysosomal enzymes normally responsible for the breakdown of glucosaminoglycans results in an accumulation and deposition of undegraded or partially degraded glucosaminoglycans in the lysosomes of many tissues Mucopolysaccharidoses (MPS) are a group of rare lysosomal storage disorders characterized by the accumulation of glycosaminoglycans (GAGs) in different parts of the eye. Ocular problems are very common in MPS children, and the cornea, sclera, trabecular meshwork, retina, and optic nerve may all be involved. Early diagnosis is very important to preserve the visual function, and the diagnosis. Mucopolysaccharidosis type VI (MPS VI), also known as Maroteaux-Lamy syndrome, is a progressive condition that causes many tissues and organs to enlarge, become inflamed or scarred, and eventually waste away (atrophy). Skeletal abnormalities are also common in this condition. The rate at which symptoms worsen varies among affected individuals Background: Mucopolysaccharidosis type I (MPS I) results in significant disease burden and early treatment is important for optimal outcomes. Recognition of short stature and growth failure as symptoms of MPS I among pediatric endocrinologists may lead to earlier diagnosis and treatment
. It has an early age of onset with clinical symptoms involving multiple organ systems. The severity of the disease depends on the phenotype. The severe phenotype has high morbidity and mortality. Early diagnostic evaluation should be carried out in suspected. Symptoms of mucopolysaccharidosis type II. Children with MPS II often have no symptoms of the condition at birth. People with severe MPS II generally begin to show signs and symptoms of the disorder between 18 months and 4 years of age. Symptoms of slowly progressive forms of MPS II tend to present a couple of years later Mucopolysaccharidosis Symptoms and Causes: causes - General: Mucopolysaccharidosis (MPS) is a group of diseases that are also classified as lysosomal storage diseases. Lysosomes are compartments in the cell that contain various enzymes that degrade (break down) molecules. In MPS, glycosaminoglycans or mucopolysaccharides are not degraded Symptoms of mucopolysaccharidosis. Symptoms include mucopolysaccharidosis stunting, which begins at the end of the first year of a child's life. It should be noted coarse facial features, large tongue, overhanging forehead, ears and teeth deformity, hypertelorism. Deformed chest, pronounced kyphosis of the lumbar and thoracic spine
Mucopolysaccharidosis Type 2 is a progressive genetic disorder. Children born with the syndrome are usually healthy at birth, and symptoms usually develop from the age of 2 years or slightly older. The disease has two types: severe and mild, with varying degrees of symptoms between the types Mucopolysaccharidosis: One of a series of inherited metabolic disorders affecting a type of complex carbohydrate called a mucopolysaccharide that is deposited in body tissues because the person lacks the specific enzyme needed to metabolize it. The deposition of mucopolysaccharide in tissues damages and distorts them, stunts the child's growth and development, limits their joint movement and. Symptoms of Mucopolysaccharidoses in Dogs. Symptoms vary widely between the different types of Mucopolysaccharidoses. The most common systems can include: Slow growth. Unusually broad chest. Large head. Dwarfism. Corneal clouding and/or ocular lesions. Weakness in the hind legs and/or deformed legs Hurler syndrome is the most severe form of mucopolysaccharidosis type 1 (MPS type 1), a rare lysosomal storage disease, characterized by skeletal abnormalities, cognitive impairment, heart disease, respiratory problems, enlarged liver and spleen, characteristic facies and reduced life expectancy. Hurler syndrome is caused by the absence of. Hunter syndrome is a very rare, inherited genetic disorder caused by a missing or malfunctioning enzyme. In Hunter syndrome, the body doesn't have enough of the enzyme iduronate 2-sulfatase. This enzyme's job is to break down certain complex molecules, and without enough of this enzyme, the molecules build up in harmful amounts. The buildup of.
Enzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI. Cochrane Database Syst Rev 2016; 3:CD009806. Giugliani R, Lampe C, Guffon N, et al. Natural history and galsulfase treatment in mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome)--10-year follow-up of patients who previously participated in an MPS VI Survey Study Mucopolysaccharidosis (MPS) involves defective activity of the lysosomal enzymes that degrade mucopolysaccharides (glycosaminoglycans [GAGs] attached to a link protein with a hyaluronic acid core) into smaller components.  The resulting incomplete degradation process leads to abnormal accumulation of heparan sulfate, dermatan sulfate, and keratan sulfate, and the abnormal accumulation of. The diagnosis of mucopolysaccharidosis should be considered for individuals with suggestive joint symptoms without signs of inflammation, particularly when other clinical signs are present (such. Hurler syndrome, also known as mucopolysaccharidosis Type IH (MPS-IH), Hurler's disease, and formerly gargoylism, is a genetic disorder that results in the buildup of large sugar molecules called glycosaminoglycans (GAGs) in lysosomes.The inability to break down these molecules results in a wide variety of symptoms caused by damage to several different organ systems, including but not limited.
Mucopolysaccharidosis (MPS) are a group of inborn metabolic disorders due to the absence or malfunctioning of specific enzymes required to process molecules called glycosaminoglycans Mucopolysaccharidosis type I (MPS I) is a rare disease in which the body is missing or does not have enough of an enzyme needed to break down long chains of sugar molecules. These chains of molecules are called glycosaminoglycans (formerly called mucopolysaccharides) Early symptoms for Mucopolysaccharidosis I (MPS I), especially in those with the attenuated form of the disease, can be very difficult to distinguish from other more common conditions. Since this progressive disease has a treatment that can slow progression, it is imperative that clinicians recognize symptoms early so a correct diagnosis can be.
Based on the severity of symptoms, mucopolysaccharidosis type I (MPS 1) can be divided into three major clinical entities - Hurler and Scheie syndromes represent phenotypes at the two different. What are the Aigns and Symptoms of Mucopolysaccharidosis Type IV? Mucopolysaccharidosis Type IV is a congenital disorder; however, noticeable features of the disorder are not commonly present at birth. The onset of significant signs and symptoms occur during early childhood (by age 2 years) Mucopolysaccharidosis Type IIIA is a rare genetic metabolic disorder. It is often also referred to as Sanfilippo syndrome. The disease usually presents itself post infancy in early childhood, and a major symptom is a developmental regression
The undegraded GAGs usually leak out of the cells and can be detected in the urine, which serves as a screening test. The common clinical signs include growth retardation, skeletal deformities, corneal cloudiness, facial dysmorphia and, in some cases, neurological signs. MPS disorders have been identified in both dogs and cats (see Table ) Mucopolysaccharidosis type II (MPS II) is one of a group of inherited (genetic) conditions that prevents the body from processing sugars properly. Mucopolysaccharidosis Type I is another condition in this group. Symptoms may appear as early as the first year of life or not until several years of age. Signs of the condition may include the. Mucopolysaccharidosis I (MPS1 for Basset Hounds Only) MPS1 is a type of lysosomal storage disease, where the lysosomes do not perform their usual function of cleaning out the garbage in a cell, but instead the waste is stored. With time, the cell can no longer function, and clinical signs of disease appear The signs and symptoms of mucopolysaccharidosis I (MPS I) are not present at birth, but they begin to appear during childhood. People with severe MPS I develop the features of this condition earlier than those with attenuated MPS I. The following list includes the most common signs and symptoms of MPS I: Enlarged head, lips, cheeks, tongue, and.
Symptoms of mucopolysaccharidosis type I. Mucopolysaccharidosis, type IH (Hurler syndrome) In patients with the Hurler syndrome, the first clinical signs of the disease appear in the first year of life, with a peak of manifestation from 6 to 12 months. In a number of cases, a slight increase in the liver, umbilical or inguinal and scrotal. Mucopolysaccharidosis type I (MPS I) is caused by the deficiency of the lysosomal enzyme α-L-iduronidase. In the severe form (Hurler disease) skeletal deformities and a delay in motor and mental development are the leading symptoms. Radiological examination of the skeleton reveals a characteristic pattern that is called dysostosis multiplex Hunter syndrome, or mucopolysaccharidosis type II (MPS II), is a member of a group of inherited metabolic disorders collectively termed mucopolysaccharidoses (MPSs). The MPSs are caused by a deficiency of lysosomal enzymes required for the degradation of mucopolysaccharides or glycosaminoglycans (GAGs) Severe neurological symptoms like Sanfilippo syndrome and hunter syndrome are some of the primary symptoms of mucopolysaccharidosis and key players are involved in the research and development of advanced therapeutics for managing these symptoms
This causes a wide range of symptoms, including behavioural problems, learning disabilities and eventually patients' ability to walk. The disease is usually diagnosed in children between two and six years of age. Mucopolysaccharidosis type III is a seriously debilitating and life-threatening disease that progresses to serious mental disability Hong Kong Global Medicine Trading Co., Ltd., approved by the Hong Kong Government and approved by the Hong Kong Department of Health, provides you with professional information on the symptoms, treatments and medicines of mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome). Question of mucopolysaccharidosis type IVA (MPS IVA; Morquio A syndrome) Mucopolysaccharidosis type I, or MPS I, is a rare genetic metabolic disorder caused by deficiency of a lysosomal enzyme required to break down mucopolysaccharides. The disorder presents as a spectrum ranging from severe forms, classically known as Hurler syndrome, which are associated with life-threatening complications, to attenuated forms.
Mucopolysaccharidosis type I (MPS I) is caused by the deficiency of α-l-iduronidase, leading to the storage of dermatan and heparan sulfate. There is a broad phenotypical spectrum with the presence or absence of neurological impairment. The classical form is known as Hurler syndrome, the intermediate form as Hurler-Scheie, and the most attenuated form is known as Scheie syndrome MPS VI (mucopolysaccharidosis VI), also known as Maroteaux-Lamy Syndrome, is an inherited lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine 4-sulfatase (arylsulfatase B), an enzyme normally required for the breakdown of certain complex carbohydrates known as glycosaminoglycans (GAGs) mucopolysaccharidoses: Definition Mucopolysaccharidosis (MPS) is a general term for a number of inherited diseases that are caused by the accumulation of mucopolysaccharides, resulting in problems with an individual's development. With each condition, mucopolysaccharides accumulate in the cells and tissues of the body because of a deficiency. Mucopolysaccharidosis (MPS) and oligosaccharidosis are lysosomal storage disorders (LSDs) that share many clinical features. The present study aimed to establish a protocol for the biochemical diagnosis of these disorders and their subtypes in affected Egyptian children as well as in pregnant females, in order to prepare children or fetus for enzyme replacement therapy Mucopolysaccharidosis type I is a genetic condition characterized by the body's inability to break down complex sugar molecules called glycosaminoglycans. As the GAGs accumulate, disease symptoms become debilitating and life threatening. Disease severity and onset of symptoms seen in MPS I are highly variable and affect many different parts.
Mucopolysaccharidosis I-Hurler (MPS I-H) is the most severe form of a metabolic genetic disease caused by mutations of IDUA gene encoding the lysosomal α-L-iduronidase enzyme. MPS I-H is a rare, life-threatening disease, evolving in multisystem morbidity including progressive neurological disease, upper airway obstruction, skeletal deformity and cardiomyopathy Mucopolysaccharidosis type VII (MPS VII) is a rare genetic metabolic disorder. What causes it? People with MPS VII do not produce enough beta-glucuronidase, an enzyme that plays a key part in the breakdown off specific sugars in the body Vimizim ® (elosulfase alfa) is the first approved enzyme replacement therapy designed to address the underlying cause of Morquio A syndrome, or mucopolysaccharidosis IVA (MPS IVA) — a deficiency in the enzyme N-acetylgalactosamine-6 sulfatase (GALNS). VIMIZIM works at a cellular level to help with deficient enzyme activity. Morquio A is a rare and progressive inherited disease that affects.
Mucopolysaccharidosis type II (MPS II) is a rare disease in which the body is missing or does not have enough of an enzyme needed to break down long chains of sugar molecules. These chains of molecules are called glycosaminoglycans (formerly called mucopolysaccharides) Mucopolysaccharidosis type VII (MPS VII), also known as Sly syndrome, caused by deficiency of the lysosomal enzyme β-glucuronidase, is an ultra-rare disorder with scarce epidemiological data and few publications about natural history and clinical spectrum. We conducted a case series report which included retrospective data from all MPS VII patients diagnosed through the MPS Brazil Network. Introduction. Mucopolysaccharidosis VI (MPS VI), or Maroteaux-Lamy syndrome (OMIM 253200) is a rare genetic disease first described in 1963 by the French doctors Pierre Maroteaux and Maurice Lamy. 1 The disease is inherited as an autosomal recessive trait and is caused by mutations in the ARSB gene, that encodes the lysosomal enzyme E.C.126.96.36.199 or ASB.
Key words: mucopolysaccharidosis, enzyme replacement therapy, mutations, glycosaminoglycans. Received: July 8, 2010; Accepted: March 23, 2011. The mucopolysaccharidoses (MPS) are a rare group 2006, identified 161 MPS patients, among which MPS II of lysosomal storage diseases (LSD) with an autosomal re- showed the highest incidence (n = 82) and. Respiratory symptoms are commonly seen in individuals with mucopolysaccharidosis (MPS) and may lead to an MPS diagnosis. Airway obstruction and respiratory impairment are prominent features of MPS and may progress early in the course of the disease. 1 Respiratory disorders are prevalent across most MPS types and have a variety of manifestations, including narrow airways and sleep-disordered. The mucopolysaccharidoses are a group of inherited metabolic diseases in which a defective or missing enzyme causes large amounts of complex sugar molecules to accumulate in harmful amounts in the body's cells and tissues. This accumulation causes permanent, progressive cellular damage that affects appearance, physical abilities, organ and system functioning, and, in most cases, mental. MPS III is the most common form of mucopolysaccharidosis, and 1 in 70,000 newborns are born with the disease. MPS III A and MPS III B are more common than MPS III C and MPS III D. Symptoms often appear after age 1, and learning abilities begin to slow between ages 2 and 6. Normal growth continues during the first few years, but will begin.
Sanfilippo syndrome, also known as mucopolysaccharidosis type III (MPS III), is a rare, inherited disorder. Sanfilippo syndrome is classified as a lysosomal storage disorder (LSD). In these disorders, genetic variations disrupt the normal activity of lysosomes in human cells MPS I (Hurler-Scheie) is a continuum of severity based upon the symptoms, ranging from severe to attenuated. There is a great deal of variability of symptoms among individuals with MPS I, often making the specific designation difficult. Generally, severe MPS I will present within the first year of life while less severe (attenuated) forms. There is no curative treatment for any form of mucopolysaccharidosis. MPS diseases are progressive and usually life-limiting (Table). In the absence of an actual cure, the symptoms are treated instead. Examples of symptomatic treatment include hernia repair, surgical decompression of the spinal cord, removal of the tonsils and adenoids, ear tub MPS VI (mucopolysaccharidosis VI), also known as Maroteaux-Lamy Syndrome, is an inherited lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine 4-sulfatase (arylsulfatase B), an enzyme normally required for the breakdown of certain complex carbohydrates known as glycosaminoglycans (GAGs)
Causes. Diagnosis. Treatment. Coping. A rare genetic metabolic disorder, Morquio syndrome is a condition in which the body is unable to process certain types of sugar molecules (glycosaminoglycans). Also known as mucopolysaccharidosis type IV (MPS IV), this disease expresses as a predominantly skeletal disorder, leading to an abnormal curvature. Symptoms of mucopolysaccharidosis II . The clinical phenotype is extremely heterogeneous and rather conditionally subdivided into a heavy and light form, representing in fact a continuum of clinical phenotypes that differ in severity. In patients with severe mucopolysaccharidosis II, clinical symptoms similar to those of Hurler's syndrome are. Morquio syndrome is an inherited metabolic disorder that affects the skeleton. It is defined by the body's inability to break down large sugar chains called glycosaminoglycans Mucopolysaccharidosis Das MAHE Usha Madhuri Nandhini 2. Symptoms • By one year of age signs and symptoms of the disorder become apparent. • Affected systems include skeletal, neurologic, cardiovascular, hearing, digestive, vision and respiratory. • Symptoms can be severe including joint deformities, deafness, blindness and significant. Test description. The Invitae Mucopolysaccharidoses Plus (MPS +) Panel analyzes genes associated with mucopolysaccharidoses, mucolipidoses, and oligosaccharidoses.This panel may be appropriate for individuals with signs and symptoms of mucopolysaccharidosis, such as coarse facial features, progressive cognitive disability, inguinal and/or umbilical hernias, hepatosplenomegaly, cardiac valve.
Hunter syndrome (mucopolysaccharidosis II) is a rare X-linked lysosomal storage disease caused by deficiency of the enzyme iduronate-2- sulfatase Baker E, Guo XH, Orsborn AM, et al. The morquio A syndrome (mucopolysaccharidosis IVA) gene maps to 16q24.3. Am J Hum Genet 1993; 52:96. Northover H, Cowie RA, Wraith JE. Mucopolysaccharidosis type IVA (Morquio syndrome): a clinical review. J Inherit Metab Dis 1996; 19:357. Mikles M, Stanton RP. A review of Morquio syndrome Autistic symptoms and mucopolysaccharidosis III, Type A. Kyle Delaney. Abstracts / Molecular Genetics and Metabolism 102 (2011) S3-S47 S39 Autistic symptoms and mucopolysaccharidosis III, Type A ARSA -/- mice. All mice tolerated both the surgical procedure and the presence of hARSA, LV-eGFP and LV-hARSA without adverse events Mucopolysaccharidosis type I (MPS I) is an inherited condition that affects many different parts of the body. It is considered a lysosomal storage disorder because individuals with MPS I have lysosomes (the recycling center of each cell) that cannot break down certain types of complex sugars. This causes undigested sugar molecules and other.
Mucopolysaccharidosis type IV Definition. Mucopolysaccharidosis type IV (MPS IV) is a rare disease in which the body is missing or does not have enough of an enzyme needed to break down long chains of sugar molecules. These chains of molecules are called glycosaminoglycans (formerly called mucopolysaccharides) Barbara Burton, MD, of Northwestern University Feinberg School of Medicine, provides an overview of the various mucopolysaccharidosis (MPS) conditions.. The MPSs are a group of lysosomal storage disorders that include MPS I (e.g., Hurler syndrome), MPS II (Hunter syndrome), MPS III (Sanfilippo syndromes), MPS IV (Morquio syndrome), MPS VI (Marateaux-Lemy syndrome) and MPS VII (Sly syndrome) So there are actually fourteen different classifications of Mucopolysaccharidosis. Hunter's Syndrome (Type II) is the only one that is linked to a genetic chromosome instead of being autosomal recessive (Mucopolysaccharidoses Types I-VII, 2014) Mucopolysaccharidosis I (Hurlers Syndrome) Introduction. MPS I (Hurler Syndrome) is an autosomal recessive mucopolysaccharide storage disorder caused by a deficiency of the lysosomal enzyme a-iduronidase. This deficiency results in the accumulation of heparan and dermatan sulphates in the lysosome
Hunter syndrome at a glance. Also known as mucopolysaccharidosis type II (MPS II) X-linked recessive genetic condition affects 1 in 100,000 to 1 in 170,000 males worldwide 1. Children typically diagnosed between ages 2-6. Caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase (IDS) resulting from a change in the IDS gene Mucopolysaccharidosis Type III (Sanfilippo Syndrome): 52 MPS IIIA, patients with onset of signs and symptoms in early childhood may have a more 53 rapidly progressive cours
Mucopolysaccharidosis type IIIB (MPS IIIB), also known as Sanfilippo syndrome Type B, is caused by harmful changes (mutations) in the NAGLU gene. The symptoms associated with MPS IIIB are caused by a buildup of harmful substances in the central nervous system and cause progressive destruction of nerve cells Symptoms of Mucopolysaccharidosis type VII Symptoms : The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database
Mucopolysaccharidosis (MPS) is a group of rare genetic disorders characterized by a deficiency of lysosomal enzymes responsible for the normal degradation of glycosaminoglycans (GAGs). The accumulation of GAGs in the lysosomes of cells is the underlying cause of the symptoms of MPS Mucopolysaccharidosis type II, or Hunter syndrome, is a rare genetic disorder. Most cases are in males. It happens when the enzyme needed to break down complex sugars is missing or not working as it should. Enzymes are proteins that speed up chemical activity in the cells. More to Kno Ocular pathology is common in patients with mucopolysaccharidosis (MPS), a hereditary lysosomal storage disorder, where the eye as well as other tissues accumulate excessive amounts of glycosaminoglycans. Despite genetic and phenotypic heterogeneity within and between different types of MPS, the disease symptoms and clinical signs often manifest during the first 6 months of life with. What are the main symptoms of mucopolysaccharidosis type III? Patients with MPS III usually appear normal at birth. Children may meet milestones up until age 2-6 and then start showing abnormal behaviors that get worse as the child gets older. At approximately 3-5 years of age most patients begin to develop mental and motor developmental delay. Sanfilippo syndrome, also known as mucopolysaccharidosis type III (MPS III), is a rare genetic disease that most of the population's unaware of. For this reason, and to give visibility to people suffering from this syndrome, we've decided to write the following article
The Mucopolysaccharidosis type VI (MPS VI), also known as Maroteaux-Lamy syndrome (OMIM 253200) is an autosomal recessive lysosomal disorder, caused by the deficiency of the enzyme N-acetylgalactosamine 4-sulfatase (also known as arylsulfatase B) due to mutations of the ARSB gene. Cardiologic features are well recognized, and are always present in MPS VI patients Objective: Mucopolysaccharidosis I (MPS I) is a progressive, debilitating, and life-threatening genetic disease, which, owing to the nonspecific nature of the early symptoms, is often unrecognized and associated with significant diagnostic delays
Hurler syndrome is the most severe type of mucopolysaccharidosis. Symptoms can range from mild to severe. Symptoms of Hurler syndrome most often appear between ages 3 and 8. Some individuals have skeletal and joint deformities that affect mobility We present a very rare case of mucopolysaccharidosis with atypical presentation such as mild mental retardation, an acrocephalic head and no corneal clouding. The purpose of presenting this case is to highlight the distinctive manifestation of mucopolysaccharidosis type II (Hunter syndrome). A 10-year-old East Asian boy presented with abdominal distension of five years' duration and complained. Mucopolysaccharidosis Type I (MPS I)is an inherited condition in which the body lacks an enzyme called alpha-L-iduronidase. Without this enzyme, the body cannot properly break down long chains of sugar molecules called glycosaminoglycans. As a result, these molecules accumulate in the body, causing numerous health problems
Some symptoms (hernia, macrocephaly, respiratory infections, and limited hip abduction) become apparent early in infancy but the complete clinical picture develops during the second year of life.mucopolysaccharidosis (mps) i-s synonyms: scheie syndrome late hurler syndrome forme fruste of hurler syndrome ullrich-scheie syndrome spat-hurler. Previously, we reported a novel disease of impaired glycosaminoglycans (GAGs) metabolism without deficiency of known lysosomal enzymes—mucopolysaccharidosis-plus syndrome (MPSPS). MPSPS, whose pathophysiology is not elucidated, is an autosomal recessive multisystem disorder caused by a specific mutation p.R498W in the VPS33A gene. VPS33A functions in endocytic and autophagic pathways, but p. Mucopolysaccharidosis I is a lysosomal storage disease caused by a deficiency of α-L-iduronidase, an enzyme that cleaves the terminal α-l-idu-ronic acid residues in the glycosaminoglycans.