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Image Unavailable-BOSSBS-9631R-A680

Anti-C16orf57 Rabbit Polyclonal Antibody (Alexa Fluor® 680)

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
Image Unavailable-BOSSBS-13147R

Anti-FNBP1 Rabbit Polyclonal Antibody

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
Image Unavailable-BOSSBS-13147R-CY3

Anti-FNBP1 Rabbit Polyclonal Antibody (Cy3®)

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
Image Unavailable-BOSSBS-9631R-CY5

Anti-USB1 Rabbit Polyclonal Antibody (Cy5®)

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
Image Unavailable-BOSSBS-9631R-A750

Anti-C16orf57 Rabbit Polyclonal Antibody (Alexa Fluor® 750)

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
Image Unavailable-BOSSBS-9631R-CY7

Anti-USB1 Rabbit Polyclonal Antibody (Cy7®)

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
Image Unavailable-BOSSBS-9631R-A488

Anti-USB1 Rabbit Polyclonal Antibody (Alexa Fluor® 488)

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
Image Unavailable-BOSSBS-13147R-A647

Anti-FNBP1 Rabbit Polyclonal Antibody (Alexa Fluor® 647)

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
Image Unavailable-BOSSBS-13147R-A680

Anti-FBP17 Rabbit Polyclonal Antibody (Alexa Fluor® 680)

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
Product Image-RPBS04-854

Respirators, industrial, PX5 PAPR Series

Supplier: RPB SAFETY
Description: The RPB PX5 is a battery powered air purifying respirator fan unit which draws in contaminated air from the wearer's immediate vicinity, passing it through internal filters, and supplying the filtered air to an approved RPB loose fitting respirator head top.

Product Image-RPBS04-900

SAR Breathing airline filters, 2 outlet, Radex

Supplier: RPB SAFETY
Description: The RPB radex 2 outlet breathing air filter is designed to filter compressed breathing air of contaminants such as particulates down to 0,15 µm, odors, tastes, and moisture, in accordance with EN 12021 requirements.

Image Unavailable-BOSSBS-9631R

Anti-C16orf57 Rabbit Polyclonal Antibody

Catalog Number: (BOSSBS-9631R)
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
Image Unavailable-BOSSBS-13147R-CY5

Anti-FNBP1 Rabbit Polyclonal Antibody (Cy5®)

Catalog Number: (BOSSBS-13147R-CY5)
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
Image Unavailable-BOSSBS-13147R-CY7

Anti-FNBP1 Rabbit Polyclonal Antibody (Cy7®)

Catalog Number: (BOSSBS-13147R-CY7)
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
Image Unavailable-BOSSBS-9631R-HRP

Anti-USB1 Rabbit Polyclonal Antibody (HRP (Horseradish Peroxidase))

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
Product Image-RPBS08-420-03

Gas monitor for supplied breathing air, GX4

Supplier: RPB SAFETY
Description: The RPB GX4 gas monitor continually monitors the quality of supplied breathing air. As soon as the air is no longer of EN breathing air standard, the unit will alarm to warn the respirator wearer that air entering the respirator is no longer safe to breath.

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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 on +353 1 88 22222.
This product is marked as restricted and can only be purchased by approved Shipping Accounts. If you need further assistance, email VWR Regulatory Department at eurega_services@eu.vwr.com
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The original product is no longer available. The replacement shown is available.
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