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Catalog Number: (BOSSBS-13147R-A555)
Supplier: Bioss
Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-A680)
Supplier: Bioss
Description: Involvement in disease; Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterised by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-CY3)
Supplier: Bioss
Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-A647)
Supplier: Bioss
Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-13147R-CY3)
Supplier: Bioss
Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-13147R)
Supplier: Bioss
Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-CY5)
Supplier: Bioss
Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-A750)
Supplier: Bioss
Description: Involvement in disease; Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterised by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-CY7)
Supplier: Bioss
Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-A488)
Supplier: Bioss
Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-13147R-A647)
Supplier: Bioss
Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-13147R-A680)
Supplier: Bioss
Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
UOM: 1 * 100 µl


Supplier: WTW
Description: OxiTop®-IDS (Intelligent Digital Sensors) is a modern measuring instrument for use in all kinds of respirometric studies. The versatile OxiTop®-IDS is suitable for both aerobic or anaerobic examinations. All heads can be used independently from any meter for normal BOD measurements between one and seven days.

Catalog Number: (BOSSBS-9631R-A350)
Supplier: Bioss
Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-HRP)
Supplier: Bioss
Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


Catalog Number: (BOSSBS-9631R-A555)
Supplier: Bioss
Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM: 1 * 100 µl


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Stock for this item is limited, but may be available in a warehouse close to you. Please make sure that you are logged in to the site so that available stock can be displayed. If the call is still displayed and you need assistance, please call us at +43 1 97002 - 0.
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