Classification of amyloidosis.
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Amyloid↗
Biomedical subjects
G Husby
Publications and source records attributed to G Husby.
Proteoglycans, glycosaminoglycans and amyloid deposition.
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Amyloid↗
The primary structure of serum amyloid A protein in the sheep: comparison with serum amyloid A in other species.
Serum amyloid A (SAA) protein was isolated from acute phase sheep sera by ultracentrifugation, gel filtration and ion-exchange chromatography. The purified protein was characterized by sodium dodecylsulfate polyacrylamide gel electrophoresis (SDS-PAGE), isoelectric focusing, amino acid composition and Edman degradation. Protein SAA sheep consists of 112 amino acid residues and has a blocked N-terminus. The amino acid sequence showed a high degree of homology with SAA proteins from other species, especially at positions 32 to 54, indicating that this particular part of the protein is important for its function. When compared to human protein SAA, nine inserted amino acids could be demonstrated, located in regions 69 to 77. Similar observations have been seen in cow, horse, dog, cat, and mink protein SAA. Heterogeneities were found in positions 28, 55, 63, 64, 66, 75, 77, 78, 80 and 89. Positions 63, 64, 66, 75, 77, 78 and 80 revealed the existence of a minor gene product of protein SAA sheep. The minor variant of protein SAA sheep is identical in these positions with the corresponding positions in protein SAA cow. By comparing the amino acid sequences of the different SAA proteins, two separate branches in the evolutionary pattern of protein SAA appear. One of the branches includes the species with the insertion which represents also one of the more heterogeneous part of the protein.
Acute-Phase Reaction↗
Bovine glomerular amyloidosis: morphological studies.
Bovine kidney material with advanced glomerular deposits of amyloid was studied immunohistochemically using the avidin biotin complex immunoperoxidase method, with rabbit anti-horse AA serum as primary antibody. Severely affected glomeruli consisted of strongly reacting positive material, obscuring all cellular structures. Transmission electron microscopy revealed that the amyloidotic areas, with evident amyloid fibrils, also contained a considerable admixture of cellular remnants. In this investigation it was found that such material was more abundant in the bovine glomerular amyloid masses than in amyloid laden organs from other animal species, and it is discussed to what extent this observation may explain the varying tinctorial properties of amyloid deposits in bovine tissues, and the relative high content of histones in bovine amyloid proteins.
Amyloidosis↗
Autonomic function in systemic lupus erythematosus.
A controlled study with paired analysis of data was performed in 34 patients with systemic lupus erythematosus (SLE) and 34 age and sex-matched healthy controls. Autonomic function was not affected significantly in SLE patients as judged by standardised cardiovascular tests. Pancreatic polypeptide (PP) response to meal stimulation, which is impaired in parasympathetic failure, was for unknown reasons found to be significantly increased in SLE patients both in unstimulated and stimulated states. Neither cardiovascular nor serological tests could thus reveal significant autonomic dysfunction in SLE. Drugs with cardiovascular effects highly influenced autonomic function and could thus be misinterpreted as autonomic dysfunction caused by SLE itself.
Adolescent↗
Effect of cimetidine on gastrointestinal symptoms in patients taking nonsteroidal anti-inflammatory drugs. A large double-blind placebo controlled study.
The effect of cimetidine on gastrointestinal (GI) symptoms in patients taking non-steroidal anti-inflammatory drugs was studied in a multicentre, double-blind, placebo-controlled 4 week trial. Five hundred and seventy osteoarthritis patients received cimetidine 400 mg bid or placebo. Seventy-nine % in the cimetidine group had no GI symptoms at week 4 compared to 72% in the placebo group (p = 0.07). Only 5.6% in the cimetidine group reported heartburn compared to 19.2% in the placebo group. Diarrhoea was more pronounced in cimetidine treated patients; 18.5% compared to 2.7%. In a subgroup of patients with previous GI discomfort on NSAID therapy (N = 123), 63% reported disappearance of symptoms in the cimetidine group compared to 45% in the placebo group (p = 0.05). In patients with GI symptoms starting NSAID therapy and in patients on NSAID with a heartburn problem, cimetidine seems to be of value.
Adult↗
Expression of serum amyloid A genes in mink during induction of inflammation and amyloidosis.
Serum amyloid A (SAA) is an acute phase protein and the precursor of amyloid protein A (AA) in deposits of secondary amyloidosis. Several isotypes exist in mink, but previous studies suggest that mink AA is derived from only one. To assess the effect of repeated episodes of inflammation and induction of amyloidosis, qualitative and quantitative changes in hepatic and extrahepatic SAA mRNA were studied. Young female mink received subcutaneous lipopolysaccharide injections for amyloid induction. Studies were performed using RNA probes and oligonucleotide probes specific for each of two SAA mRNA species. Northern blot hybridization showed that hepatic SAA1 and SAA2 mRNA levels increased dramatically after inflammatory stimulation, and were subsequently maintained at elevated levels, showing considerable interindividual variation, but only a slight decrease during repeated inflammatory stimuli and the early stages of amyloid deposition. No preferential accumulation of mRNA specifying a particular isotype was found during the experiment. Differential expression of mink SAA mRNA during repeated inflammatory stimulation does not seem to explain why only SAA2-derived AA is found in amyloid deposits. Extrahepatic SAA mRNA seemed to be independently regulated and may thus represent another, yet not characterized, SAA isotype.
Amyloidosis↗
Retrospective molecular detection of Transthyretin Met 111 mutation in a Danish kindred with familial amyloid cardiomyopathy, using DNA from formalin-fixed and paraffin-embedded tissues.
Severe familial amyloid cardiomyopathy (FAC) in a Danish kindred is associated with a specific mutation (Met for Leu 111) in the transthyretin (TTR) gene. The mutation causes the loss of a DdeI restriction site in the gene, allowing molecular diagnostic studies. We studied formalin-fixed, paraffin-embedded tissues, up to 39 years old, from 29 family members of this kindred. DNA was partially purified from deparaffinized tissue sections and a DNA sequence of the TTR gene flanking the mutation site was amplified by the polymerase chain reaction (PCR), followed by restriction enzyme analysis. Amplified DNA was obtained from tissues representing 23 of the 29 persons. Ten out of the 23 family members were found to carry the TTR Met 111 mutation, whereas 13 were not affected. The results were consistent with known clinical data and with corresponding serum TTR examinations. This retrospective study shows that archival tissues can be used to confirm the diagnosis and disease pattern in members of families affected by hereditary diseases.
Adolescent↗
[Quality of an outpatient rheumatology clinic. Consumers' evaluation of medical aspects and service].
As part of our quality assurance programme we designed special questionnaires which addressed expectations concerning medical aspects and service at our out-patient rheumatology clinic, and the degree of satisfaction. These questionnaires were answered by 97 patients, 86 referring general practitioners and five rheumatologists. The principle purpose of the consultation with the specialist, both for patients and referring practitioners, was to obtain or confirm the diagnosis. 76% of the patients and 91% of the general practitioners were satisfied with the medical aspect of the consultation. The dissatisfied patients were mainly those with symptomatic diagnoses (pain syndromes) not explained by objective changes. As for the service, long waiting time before consultation or before receiving the specialist's report was the main reason for dissatisfaction among patients and referring practitioners respectively.
Adult↗
Characterization and quantification of mink serum amyloid A protein using two-dimensional electrophoresis with immobilized pH gradients.
Using hydrophobic interaction chromatography, two-dimensional electrophoresis with an immobilized pH gradient in the first dimension and semidry blotting, three isoforms of mink serum amyloid A protein (SAA) were characterized and studied during chronic inflammation. Compared to conventional methods that have been applied to SAA, the major advantages of the present combination of methods are: (i) use of small serum volumes, (ii) rapid extraction, (iii) high resolution, and (iv) high yield of proteins.
Acute-Phase Reaction↗
The epidemiology of ankylosing spondylitis.
This review focuses on various epidemiological aspects of ankylosing spondylitis (AS). Diagnostic criteria currently available are described, and their use in scientific studies as opposed to everyday clinical practice is discussed. Present knowledge of the prevalence of AS is addressed in detail with particular emphasis on discrepancies caused by variations in population frequencies of HLA B27 and those caused by differences in study designs.
Adolescent↗
On the association between amyloid fibrils and glycosaminoglycans; possible interactive role of Ca2+ and amyloid P-component.
We have previously reported the specific association of glycosaminoglycans (GAG) and proteoglycans (PG) with amyloid fibrils and characterized the polysaccharides directly extracted from amyloid-laden tissues. In the present study we further elucidate the association between purified amyloid fibrils and GAG/PG with special reference to those GAG/PG associated with amyloid P-component (AP) and the interactive role of Ca2+ ions. Amyloid fibrils were isolated from human hepatic AA amyloid employing water extraction with and without preceding removal of AP, an extrafibrillar protein component of all amyloids, using sodium citrate. GAG/PG co-isolated with the amyloid extracts, with and without AP, were isolated and characterized. Agarose-affinity chromatography of extracts containing AP was performed, and the GAG associated with this extrafibrillary protein were characterized as well. Several different GAG/PG populations were demonstrated in the various extracts. The abolition of calcium-dependent binding markedly influenced the amount of GAG/PG recovered in the fibril extracts, as well as the total amount of amyloid material obtained. Thus, it seems that calcium plays an important role in the association between the fibrils and the sugar moieties, and that a significant fraction of the GAG found in amyloid exhibits a Ca(2+)-dependent fibril-GAG interaction. No significant difference in the proportion between galactosaminoglycans and glucosamines was, however, disclosed when the two extraction protocols were compared, suggesting that no particular GAG species has a higher affinity for the fibrils themselves. Both dermatan/chondroitin sulphate and heparan sulphate identified in the present study exhibited a Ca(2+)-dependent interaction with AP, supporting previous findings. However, the amyloid-associated galactosaminoglycans found, especially the large PG appearing in small amounts, seemed to have a higher affinity for the extrafibrillar AP than the other GAG.
Amyloid↗
The revised amino acid sequence of serum amyloid A (SAA) protein in mink.
The revised amino acid sequence of mink protein SAA was shown to be composed of 111 amino acid residues. The protein has an insertion of eight amino acid residues compared with that reported earlier. Microheterogeneities were observed in positions 6, 10, 24, 27, 67 and 71. The amino acid sequence is in accordance with the SAA mink cDNA sequences, except for the phenylalanine in position 6. The data indicate a third SAA gene in mink.
Amino Acid Sequence↗
Purification of amyloid-associated heparan sulphate proteoglycans and galactosaminoglycan free chains from human tissues.
Basement membrane-associated heparan sulphate proteoglycans have been demonstrated immunohistochemically in organs from patients afflicted with various types of amyloidosis. In a recent report, we were able to isolate and partly characterize a basement membrane-associated heparin sulphate proteoglycan from human hepatic amyloid. In the present study proteoglycans were extracted with guanidine from human amyloid-laden kidney, spleen and lymph nodes. All tissues extracted with guanidine contained both heparan sulphate proteoglycan (HSPG) and galactosaminoglycan (CS/DS) free chains. Tissue staining using a monoclonal antibody against basement membrane HSPG revealed the presence of HSPG in amyloid deposits in kidney and spleen. Furthermore, following SDS-PAGE of HSPG from kidney after deaminative cleavage of the HS chains, a 15-kDa and 80-kDa protein appeared, probably representing the core protein(s). In lymph node HSPG, three core proteins of 65, 30 and 25 kDa could be demonstrated on SDS-PAGE, the first reacting with the anti-basement membrane HSPG antibody when subjected to Western blotting subsequent to SDS-PAGE. By immunohistochemistry, we failed to demonstrate any staining of the renal and splenic tissue sections employing an antibody against the decorin core protein.
Adult↗
Differential expression of rabbit serum amyloid A genes in response to various inflammatory agents.
Serum amyloid A (SAA) is an acute-phase plasma protein which increases up to 1000-fold after an acute-phase stimulus. Several SAA genes and corresponding protein isotypes exist in individual species. Liver is the main source of production, but extra-hepatic SAA expression has been described. In this study inflammation was induced in rabbits with lipopolysaccharide, turpentine, or casein. Transcription of SAA mRNA was studied using Northern blot analysis with probes specific for three different rabbit SAA isotypes and analysed by scanning densitometry. In the stimulated liver slight variation in SAA mRNA transcription level was seen after stimulation with different inflammatory agents. After lipopolysaccharide-stimulation SAA gene expression was also seen in most of the extra-hepatic organs. After turpentine stimulation SAA mRNA was seen in the liver, the ovary, and the small intestines, and after casein stimulation only in the liver and the ovary. SAA1 and SAA2 were induced exclusively in the liver, while SAA3 was induced mainly in the extra-hepatic organs. This indicates that the SAA genes probably are independently regulated both in relation to stimulus, gene- and tissue-specificity.
Acute-Phase Reaction↗
The primary structure of rabbit serum amyloid A protein isolated from acute phase serum.
Serum amyloid A (SAA) protein, a sensitive acute phase protein and the precursor of protein AA in secondary amyloid, was purified from pooled acute phase rabbit serum using two different methods: isolation of protein SAA directly by octyl-Sepharose chromatography of total serum, and dissociation and isolation of apoSAA from acute phase high density lipoprotein (HDL). The protein SAA fraction obtained was further purified using gel filtration and ion exchange chromatography. Rabbit protein SAA has 104 amino acid residues, like human SAA, and has a partially blocked N terminus. The highly conserved region from position 33 to position 63 found in SAA from all species studied was confirmed also in rabbit SAA. No microheterogeneities were observed. The amino acid sequence showed extensive N-terminal homology with the rabbit amyloid A protein, except for the microheterogeneity in position 12 in protein AA. It also showed identical amino acid sequence with that deduced from the rabbit cDNA clone pSAA 55. Complete homologies were found with clone SAA 2, except for positions 22 and 78, clone SA8-1, except for positions 22 and 79 and clone SA7-3, except for position 22. This pSAA 55/SA7-3/SA8-1/SAA2-like protein was the only SAA isotype found both in total serum and in the HDL fraction. Isotypes corresponding to other SAA-like genes could not be found in this pool of acute phase rabbit sera.
Acute-Phase Reaction↗
A controlled study of peripheral neuropathy in systemic lupus erythematosus.
In 34 patients with systemic lupus erythematosus (SLE) and 34 age- and sex-matched healthy controls we performed standardized quantifiable neurological testing for neuropathic symptoms (Neuropathic Symptom Score) and deficits (Neurological Disability Score), as well as nerve conduction velocity studies (NCV) and determination of vibration thresholds (VT). SLE patients had more neuropathic symptoms and deficits than controls. Most quantitative and qualitative NCV attributes showed no difference between patients and controls. However, if categorized as NCV abnormalities of 2 or more nerves in each individual, a frequency of polyneuropathy of 21% was seen in SLE patients compared to 6% in controls. VT indicated a slight, but widespread, diffuse polyneuropathy in patients compared to controls.
Adolescent↗
Facial palsy and partial accommodative insufficiency associated with sulphasalazine treatment in a patient with ankylosing spondylitis.
We report on a patient with peripheral facial nerve palsy and blurred near vision in association with sulphasalazine treatment. The possibility of this being an adverse reaction to sulphasalazine is discussed.
Accommodation, Ocular↗