| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :1.1663791 |
| We found that cathepsin A in human placenta exists as the following three forms : a 1270 kDa complex with beta galactosidase and neuraminidase ( about 1 % of total cathepsin A ) , a 680 kDa complex with beta galactosidase ( 30 40 % of total ) , and a free 98 kDa cathepsin A dimer ( 60 70 % of total ) . 1.1663791^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.71327018 |
| GLB 1 forms a complex with protective protein cathepsin A ( PPCA ) , alpha neuraminidase ( NEU 1 ) , and galactosamine 6 sulphate sulfatase ( GALNS ) inside lysosomes , while EBP binds to PPCA and NEU 1 on the cell surface . 0.71327018^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The lysosomal storage disorder galactosialidosis results from a primary deficiency of the protective protein / cathepsin A ( PPCA ) , which in turn affects the activities of beta galactosidase and neuraminidase . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Lysosomal protective protein / cathepsin A is a serine carboxypeptidase that forms a complex with beta galactosidase and neuraminidase . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The GSL receptor for SEB was sensitive to alpha galactosidase , and resistant to beta galactosidase and beta glucosidase . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Human lysosomal beta galactosidase cathepsin A complex : definition of the beta galactosidase binding interface on cathepsin A . ^^^ Human lysosomal beta galactosidase is organized as a 680 kDa complex with cathepsin A ( also named carboxypeptidase L and protective protein ) , which is necessary to protect beta galactosidase from intralysosomal proteolysis . ^^^ To understand the molecular mechanism of beta galactosidase protection by cathepsin A , we defined the structural organization of their complex including the beta galactosidase binding interface on cathepsin A . ^^^ Radiation inactivation analysis suggested the existence of a 168 kDa structural subunit of the complex containing both beta galactosidase and cathepsin A . ^^^ Chemical cross linking of the complex confirmed the existence of this subunit and showed that it is composed of one cathepsin A dimer and one beta galactosidase monomer . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Deficiency of lysosomal protective protein / cathepsin A in humans is the primary cause of galactosialidosis , a lysosomal storage disease characterized by combined deficiency of beta galactosidase and neuraminidase . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| In the patient ' s fibroblasts , both alpha neuraminidase and beta galactosidase activities were severely reduced , and cathepsin A activity was < 1 % of control levels , confirming the biochemical diagnosis of galactosialidosis . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Association of N acetylgalactosamine 6 sulfate sulfatase with the multienzyme lysosomal complex of beta galactosidase , cathepsin A , and neuraminidase . ^^^ We show that in normal cells GALNS is in a 1 . 27 MDa complex with three other lysosomal hydrolases : beta galactosidase , alpha neuraminidase , and cathepsin A ( protective protein ) . ^^^ GALNS copurifies with the complex by different chromatography techniques : affinity chromatography on both cathepsin A binding and beta galactosidase binding columns , gel filtration , and chromatofocusing . ^^^ Anti human cathepsin A rabbit antiserum coprecipitates GALNS together with cathepsin A , beta galactosidase , and alpha neuraminidase in both a purified preparation of the 1 . 27 MDa complex and crude glycoprotein fraction from human placenta extract . ^^^ Gel filtration analysis of fibroblast extracts of patients deficient in either beta galactosidase ( beta galactosidosis ) or cathepsin A ( galactosialidosis ) , which accumulate KS , demonstrates that the 1 . 27 MDa complex is disrupted and that GALNS is present only in free homodimeric form . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The human lysosomal enzyme occurs in complex with beta galactosidase and protective protein / cathepsin A ( PPCA ) , and is deficient in two genetic disorders : sialidosis , caused by a structural defect in the neuraminidase gene , and galactosialidosis , in which the loss of neuraminidase activity is secondary to a deficiency of PPCA . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Protective protein for beta galactosidase , Ppgb , maps to the distal imprinting region of mouse chromosome 2 but is not imprinted . ^^^ PPGB , protective protein for beta galactosidase , maps to human chromosome 20q13 . 1 , and from linkage analysis of two interspecific crosses incorporating the mouse reciprocal translocations , T ( 2 ; 8 ) 2Wa ( T2Wa ) and T ( 2 ; 16 ) 28H ( T28H ) , we have mapped the mouse homologue , Ppgb , to the conserved region on distal mouse Chr 2 . ^^^ Protective protein for beta galactosidase , Ppgb , maps to the distal imprinting region of mouse chromosome 2 but is not imprinted . ^^^ PPGB , protective protein for beta galactosidase , maps to human chromosome 20q13 . 1 , and from linkage analysis of two interspecific crosses incorporating the mouse reciprocal translocations , T ( 2 ; 8 ) 2Wa ( T2Wa ) and T ( 2 ; 16 ) 28H ( T28H ) , we have mapped the mouse homologue , Ppgb , to the conserved region on distal mouse Chr 2 . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The method was used to demonstrate association between two enzymes of the mitochondrial citric acid cycle , malate dehydrogenase and citrate synthase , and between the lysosomal hydrolases , beta galactosidase and cathepsin A . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Human protective protein / cathepsin A ( PPCA ) , a serine carboxypeptidase , forms a multienzyme complex with beta galactosidase and neuraminidase and is required for the intralysosomal activity and stability of these two glycosidases . ^^^ Genetic lesions in PPCA lead to a deficiency of beta galactosidase and neuraminidase that is manifest as the autosomal recessive lysosomal storage disorder galactosialidosis . ^^^ The high incidence of PPCA folding mutants in galactosialidosis reflects the fact that a single point mutation is unlikely to affect both the beta galactosidase and the neuraminidase binding sites of PPCA at the same time to produce the double glycosidase deficiency . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Protective protein / cathepsin A ( PPCA ) is a lysosomal serine carboxypeptidase that forms a complex with beta galactosidase and neuraminidase . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Cathepsin A [ EC 3 . 4 . 16 . 1 ] , so called protective protein , occurs as an enzyme complex with lysosomal beta galactosidase [ 3 . 2 . 1 . 23 ] and is involved in the stable enzymic expression of lysosomal sialidase [ 3 . 2 . 1 . 18 ] . ^^^ In this study we investigated the enzymatic properties of cathepsin A in the bovine beta galactosidase complex and how it is involved in the molecular multiplicities of the beta galactosidase and sialidase complexes . ^^^ Immunoprecipitation using an anti beta galactosidase antibody demonstrated that cathepsin A is a component of both the sialidase and beta galactosidase complexes . ^^^ The 669 kDa beta galactosidase complex dissociated reversibly into a 120 kDa beta galactosidase and a 170 kDa cathepsin A , but the 120 kDa beta galactosidase , free from the cathepsin A , formed a 260 kDa aggregate under the same conditions . ^^^ Both beta galactosidase and cathepsin A activities were labile under the dissociated condition , indicating that it physiologically stabilizes not only beta galactosidase but also itself by forming the complex . . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Lysosomal sialidase occurs in a multienzyme complex that also contains beta galactosidase and cathepsin A . ^^^ Since sialidase activity requires its association with beta galactosidase and cathepsin A , the expression of mouse sialidase within human sialidosis cells underlines the structural similarity between mouse and human enzymes and suggests that the mechanism for complex formation and function is highly conserved . . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Lysosomal neuraminidase ( sialidase ) occurs in a high molecular weight complex with the glycosidase beta galactosidase and the serine carboxypeptidase protective protein / cathepsin A ( PPCA ) . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Galactosialidosis is a recessively inherited lysosomal storage disease characterized by the combined deficiency of neuraminidase and beta galactosidase secondary to the genetic deficiency of cathepsin A / protective protein . ^^^ In lysosomes , cathepsin A forms a high molecular weight complex with beta galactosidase and neuraminidase that protects these enzymes against intralysosomal proteolysis . ^^^ However , enzymatic assays , gel filtration , and Western blot analysis of the patient ' s cultured skin fibroblast extracts showed the presence of a small amount of normal size , catalytically active cathepsin A and cathepsin A beta galactosidase 680 kDa complex , suggesting that a low amount of cathepsin A mRNA is spliced normally and produces the wild type protein . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The acid carboxypeptidase ( cathepsin A ) and lysosomal neuraminidase activities were markedly decreased in cultured fibroblasts and chorionic villus cells derived from the patients , although the decrease in beta galactosidase activity was less . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Protective protein / cathepsin A ( PPCA ) is a pleiotropic lysosomal enzyme that complexes with beta galactosidase and neuraminidase , and possesses serine carboxypeptidase activity . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The intracellular cathepsin A , alpha N acetylneuraminidase and beta galactosidase activities , which are deficient in the parent fibroblastic cells , could also be significantly restored in the cells . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| We hypothesize that this region may be involved in the interface of sialidase binding with lysosomal cathepsin A and / or beta galactosidase in their high molecular weight complex required for the expression of sialidase activity in the lysosome . . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Cathepsin A is a multifunctional lysosomal carboxypeptidase that also functions as a protective and an activator protein for neuraminidase and beta galactosidase . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Lysosomal N Acetyl alpha neuraminidase is active in complex with the protective protein / cathepsin A ( PPCA ) and beta galactosidase . ^^^ We further demonstrated that when expressed alone , the neuraminidase precursor remained dimeric ( 114 kDa ) and had low enzymatic activity , but when co expressed with PPCA and beta galactosidase , it multimerized in a complex of approximately 1350 kDa , together with the other two proteins . ^^^ The fully active neuraminidase co precipitated with full length PPCA and beta galactosidase precursors . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The cathepsin A precursor has the potential to restore both beta galactosidase and neuraminidase activities in fibroblasts from patients with galactosialidosis . ^^^ Human lysosomal beta galactosidase and neuraminidase exist as a high molecular weight enzyme complex , in which there is a 54 kDa glycoprotein termed ' lysosomal protective protein ' . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The disintegration of the complex due to genetic mutations in its components results in their functional deficiency and causes severe metabolic disorders : sialidosis ( mutations in sialidase ) , GM 1 gangliosidosis and Morquio disease type B ( mutations in beta galactosidase ) , galactosialidosis ( mutations in cathepsin A ) , and Morquio disease type A ( mutations in N acetylaminogalacto 6 sulfate sulfatase ) . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Cathepsin A ( PPCA ) is a lysosomal carboxypeptidase that functions as a protective protein for alpha neuraminidase and beta galactosidase in a multienzyme complex . ^^^ Cathepsin A ( PPCA ) is a lysosomal carboxypeptidase that functions as a protective protein for alpha neuraminidase and beta galactosidase in a multienzyme complex . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Galactosialidosis is an autosomal recessive lysosomal storage disease caused by a combined deficiency of lysosomal beta galactosidase and neuraminidase as a result of a primary defect in the protective protein / cathepsin A ( PPCA ) . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Three acidic glycosidases : beta galactosidase ( beta GAL , EC 3 . 2 . 1 . 23 ) , alpha neuraminidase ( NEUR , sialidase , EC 3 . 2 . 1 . 18 ) , N acetylaminogalacto 6 sulfate sulfatase ( GALNS , EC 3 . 1 . 6 . 4 ) and serine carboxypepidase cathepsin A ( EC 3 . 4 . 16 . 1 ) form a functional high molecular weight complex in the lysosomes . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Cathepsin A ( PPCA ) , a lysosomal carboxypeptidase that functions as a protective protein for alpha neuraminidase and beta galactosidase in a multi enzyme complex , has been shown to be expressed in the epithelial cells of the epididymis . ^^^ Cathepsin A ( PPCA ) , a lysosomal carboxypeptidase that functions as a protective protein for alpha neuraminidase and beta galactosidase in a multi enzyme complex , has been shown to be expressed in the epithelial cells of the epididymis . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The glycosylated precursor proteins were taken up via the mannose 6 phosphate receptors , and the cathepsin A , alpha neuraminidase and beta galactosidase ( beta Gal ) activities deficient in the fibroblasts derived from a patient with PPCA deficiency ( galactosialidosis ) were restored . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| In these same cells , the specific activity of other cellular proteins ( e . g . beta galactosidase , cathepsin A and alkaline phosphatase ) increased only two to fourfold during differentiation of monocytes . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| Sialidosis and galactosialidosis are lysosomal storage diseases caused by the genetic defects of lysosomal sialidase ( neuraminidase 1 ; NEU 1 ) and lysosomal protective protein / cathepsin A ( PPCA ) , respectively , associated with a NEU 1 deficiency , excessive accumulation of sialylglycoconjugates , and development of progressive neurosomatic manifestations ; in addition , the latter disorder is accompanied by simultaneous deficiencies of beta galactosidase and cathepsin A . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| OBJECTIVE : To determine the distribution and influence of lysosomal neuraminidase ( Neul ) , protective protein / cathepsin A ( PPCA ) and beta galactosidase ( beta gal ) in the inner ear of the mouse , and to observe their auditory alterations in enzyme deficiency . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| One is a Mr 63 , 000 protein encoded by the Glb 1 gene and the other is a Mr 45 , 000 component encoded by the Glb 2 gene . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The Arabidopsis GLB 1 and GLB 2 genes are likely to be functionally distinct . ^^^ The class 2 haemoglobin gene ( GLB 2 ) is expressed in the roots , leaves and inflorescence and can be induced in young plants by cytokinin treatment in contrast to the class 1 gene ( GLB 1 ) which is active in germinating seedlings and can be induced by hypoxia and increased sucrose supply , but not by cytokinin treatment . . ^^^ |
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| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| These data confirm that PPCA is present in two functional complexes one with GLB 1 and NEU 1 in the lysosomal lumen and the other with EBP at the cell surface . ^^^ The reduction in GLB 1 and EBP confirms that PPCA is essential for their integrity . . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| The purpose of this study is to determine effects of GLB 1 , PPCA and NEU 1 gene mutations on elastogenesis in skin fibroblasts . ^^^ Apparently normal elastogenesis and EBP mRNA expression were observed for fibroblasts from Morquio B disease cases with the GLB 1 gene alleles ( W273L / W273L , W273L / R482H and W273L / W509C substitutions , respectively ) , a galactosialidosis case with the PPCA allele ( IVS7+3A / IVS7+3A ) and a sialidosis case with the NEU 1 allele ( V217M / G243R ) as well as normal subject . ^^^ |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P16278 and P10619 |
Pubmed |
SVM Score :0.0 |
| NA |
|