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Numer katalogowy: (BOSSBS-15472R-A555)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-15472R-CY3)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-15472R-CY5)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-15472R-A750)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-15472R-A350)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-8338R-A750)
Producent: Bioss
Opis: Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1); also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP). HARP is a rare syndrome with many clinical similarities to NBIA1.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-8338R)
Producent: Bioss
Opis: Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1); also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP). HARP is a rare syndrome with many clinical similarities to NBIA1.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-15472R-A647)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-15472R-A488)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-8338R-HRP)
Producent: Bioss
Opis: Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1); also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP). HARP is a rare syndrome with many clinical similarities to NBIA1.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-15472R)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyze the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterized by urine that turns dark on standing and alkalinization, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-15472R-HRP)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Numer katalogowy: (BOSSBS-15472R-FITC)
Producent: Bioss
Opis: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyse the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterised by urine that turns dark on standing and alkalinisation, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.
j.m.: 1 * 100 µl


Producent: Biotium
Opis: This antibody recognizes a ~90-95 kDa protein which is identified as cell surface transferrin receptor (CD71), a disulfide-bonded homodimeric glycoprotein of 180-190 kDa. This MAb is highly specific to CD71 and shows no cross-reaction with other related proteins. Ligand for transferrin receptor is the serum iron transport protein, transferrin. This receptor is broadly distributed in carcinomas, sarcomas, leukemias, and lymphomas. CD71/Transferrin receptor has been reported to be associated with cell proliferation in both normal and neoplastic tissues and useful in predicting clinical behavior or response to therapy in a number of malignancies including breast cancer.

Numer katalogowy: (BOSSBS-8338R-A680)
Producent: Bioss
Opis: Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1); also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP). HARP is a rare syndrome with many clinical similarities to NBIA1.
j.m.: 1 * 100 µl


Producent: Biotium
Opis: This antibody recognizes a ~90-95 kDa protein which is identified as cell surface transferrin receptor (CD71), a disulfide-bonded homodimeric glycoprotein of 180-190 kDa (Workshop IV). This MAb is highly specific to CD71 and shows no cross-reaction with other related proteins. Ligand for transferrin receptor is the serum iron transport protein, transferrin. This receptor is broadly distributed in carcinomas, sarcomas, leukemias, and lymphomas. CD71/Transferrin receptor has been reported to be associated with cell proliferation in both normal and neoplastic tissues and useful in predicting clinical behavior or response to therapy in a number of malignancies including breast cancer.

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Zapasy tego artykułu są ograniczone, ale możliwe, że jest on dostępny w pobliskim magazynie. Upewnij się, że jesteś zalogowany na stronie, aby móc sprawdzić dostępność zapasów. Jeśli call ciągle się wyświetla i potrzebujesz pomocy, zadzwoń na 58 323 82 00.
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