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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-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
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-4307R-FITC

Anti-DEFB4A Rabbit Polyclonal Antibody (FITC (Fluorescein Isothiocyanate))

Catalog Number: (BOSSBS-4307R-FITC)
Supplier: Bioss
Description: Beta-defensins (also designated BD, and hBD in human) are small cationic peptides with broad-spectrum antimicrobial activity. Produced in mucosal epithelia and neutrophils of several species, Beta-defensins are developmentally regulated. Human b-defensin 2 is locally regulated by inflammation and is the first member of the b-defensin family that is locally inducible by inflammation. The murine homolog of human b-defensin 2, which is called b-defensin 3, is present in the respiratory system and in low levels in the epithelial cells of the intestine and lung. The unique murine b-defensin 2 (Defb2) is not expressed in airways of untreated mice, but is upregulated in the airways by lipopolysaccharide and may contribute to host defense at the mucosal surface of the airways
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-RPBS08-435

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.

Image Unavailable-BOSSBS-9631R-A350

Anti-USB1 Rabbit Polyclonal Antibody (Alexa Fluor® 350)

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
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.

Product Image-RPBS17-513

Respirators, T200 Series

Supplier: RPB SAFETY
Description: The RPB T200 is a lightweight multi-purpose respirator designed specifically for the healthcare and life science industries, relieving workers of breathing difficulties and fatigue experienced with N95 use. Available with either a head harness or bump cap for head protection and multiple hood options, the T200 has the ability to adapt to different environments that need face protection and respiratory protection.

Image Unavailable-BOSSBS-9631R-A555

Anti-USB1 Rabbit Polyclonal Antibody (Alexa Fluor® 555)

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
Image Unavailable-BOSSBS-13147R-A750

Anti-FBP17 Rabbit Polyclonal Antibody (Alexa Fluor® 750)

Catalog Number: (BOSSBS-13147R-A750)
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-FITC

Anti-FNBP1 Rabbit Polyclonal Antibody (FITC (Fluorescein Isothiocyanate))

Catalog Number: (BOSSBS-13147R-FITC)
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-A350

Anti-FNBP1 Rabbit Polyclonal Antibody (Alexa Fluor® 350)

Catalog Number: (BOSSBS-13147R-A350)
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-A488

Anti-FNBP1 Rabbit Polyclonal Antibody (Alexa Fluor® 488)

Catalog Number: (BOSSBS-13147R-A488)
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
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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.
Dual use goods can only be delivered within the European Union.
Dual use goods can only be delivered within the European Union.
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