Hexosaminidase B

A mammalian beta-hexosaminidase isoform that is comprized of hexosaminidase beta subunits. Deficiency of hexosaminidase B due to mutations in the gene encoding the hexosaminidase beta subunit is a case of SANDHOFF DISEASE.

Also Known As:

Networked: 86 relevant articles (1 outcomes, 9 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Zwierz, Krzysztof: 7 articles (01/2014 - 03/2008)
2.	Waszkiewicz, Napoleon: 6 articles (11/2020 - 11/2011)
3.	Sakuraba, Hitoshi: 5 articles (01/2021 - 06/2011)
4.	Chojnowska, Sylwia: 5 articles (11/2020 - 09/2010)
5.	Szajda, Sławomir Dariusz: 5 articles (11/2020 - 09/2009)
6.	Itoh, Kohji: 4 articles (01/2018 - 06/2011)
7.	Zalewska-Szajda, Beata: 4 articles (09/2014 - 01/2012)
8.	Kępka, Alina: 4 articles (01/2014 - 11/2011)
9.	Kitakaze, Keisuke: 3 articles (09/2016 - 06/2011)
10.	Tsuji, Daisuke: 3 articles (09/2016 - 06/2011)

Related Diseases

1.	GM2 Gangliosidoses (GM2 Gangliosidosis) 06/01/2011 - "Therapeutic potential of intracerebroventricular replacement of modified human β-hexosaminidase B for GM2 gangliosidosis." 01/30/2003 - "The method is fast, precise, and highly suitable for the biochemical diagnosis and heterozygote screening of GM2 gangliosidoses, and would be applicable in cases of thermolabile Hex B and for detecting the B1 variant." 01/01/2016 - "Protease-resistant modified human β-hexosaminidase B ameliorates symptoms in GM2 gangliosidosis model." 04/01/2017 - "We report on two patients with typical clinical features suggestive of GM2 gangliosidosis, but normal results for hexosaminidase A and hexosaminidase B as well as their corresponding genes. " 09/01/2016 - "Combined replacement effects of human modified β-hexosaminidase B and GM2 activator protein on GM2 gangliosidoses fibroblasts."
2.	Neoplasms (Cancer) 10/01/1983 - "With respect to heat stability, Hex B from the normal lung was very stable at 52 degrees, while the tumor Hex B (mixture of pI 7.6 and 7.9 forms) was unstable. " 09/30/2010 - "The considerably higher activity of HEX, HEX-A, GLU, GAL, MAN, FUC and descending trend of activity of HEX-B were noticed in saliva of patients with salivary gland tumors in comparison to healthy volunteers. " 09/30/2010 - "In salivary gland tumor tissue the activity of HEX, HEX-A, HEX-B, GAL, FUC was considerably higher than in comparison to healthy salivary gland tissue and ascending trend of activity of GLU, MAN was also noticed. " 10/01/1983 - "These findings indicate that the formation of mixed disulfide bonds in the tumor Hex B increases the net negative charge and results in the appearance of a heat-labile form." 07/02/1979 - "Hex A and Hex B from the sera of cancer patients resembled their normal counterparts in heat stability, pH optimum, and apparent KM (1.07 mM for Hex A, 0.88 mM for Hex B). "
3.	Alcoholism (Alcohol Abuse) 04/01/1995 - "In the present study, we have compared "Hex B" with CDT as markers of alcohol abuse in a group of alcoholics hospitalized for detoxification after a period of heavy alcohol abuse. " 11/01/1997 - "We suggest that serum beta-Hex B% can be a useful and inexpensive laboratory test for alcohol abuse." 04/01/1995 - "The possible reasons for a relation between these two markers are discussed, and it is concluded that more experience of both "Hex B" and CDT in unselected populations is needed to establish the diagnostic potential of these tests as markers of alcohol abuse." 06/01/1994 - "Since Hex B is a sensitive marker for alcohol abuse, we also investigated its serum level in subjects that could be suspected of alcohol abuse. " 06/01/1991 - "It is concluded that Hex "B" is a useful marker for alcohol abuse."
4.	Colonic Neoplasms (Colon Cancer) 09/01/2009 - "In patients with colon adenocarcinoma mucinosum, the higher activity of HEX was revealed in blood serum compared to healthy people, and the significantly higher activity of HEX and HEX B expressed as pKat/mg of creatinine, was found in urine. " 09/01/2009 - "A significant increase in the concentration of HEX, HEX A and HEX B activity was proved in serum and urine of patients with colon adenocarcinoma. " 01/01/2012 - "The specific activity of HEX, HEX A, HEX B, GAL, FUC, MAN, and CD was assayed in serum, urine, and carcinoma tissue of 12 patients with colon adenocarcinoma. " 10/01/1984 - "In view of the reported low levels of colon cancer among Seventh-day Adventists (SDA) we determined levels of total HEX, and its heat-labile (HEX-A) and heat-stable (HEX-B) isozymes in vegetarian SDA, lacto-ovo vegetarian SDA, nonvegetarian SDA, and the general public. " 09/01/2009 - "We observe a significant increase in the activity of HEX, HEX A and HEX B expressed in pKat/mg of creatinine was found in urine of patients bearing tumor of diameter 6.0-7.0 cm in comparison to patients with tumor of diameter 4.0-5.0 cm. The present study results suggest that determination of HEX, HEX A and HEX B activity in blood serum and urine may be used to detect colon cancer in its early stages. "
5.	Sandhoff Disease (Sandhoff's Disease) 06/16/2023 - "Sandhoff disease (SD, Online Mendelian Inheritance in Man: 268800) is an autosomal recessive lysosomal storage disorder caused by variants of the β-hexosaminidase B (HEXB) gene (Online Mendelian Inheritance in Man: 606873). " 06/16/2023 - "A case of Sandhoff disease caused by a novel β-hexosaminidase B (HEXB) mutation c.118delG (p.A40fs*24): A case report from China." 01/01/2017 - "Sandhoff Disease without Hepatosplenomegaly Due to Hexosaminidase B Gene Mutation." 10/01/1997 - "Hex B (beta beta homodimer) is also defective in Sandhoff disease. " 09/01/1993 - "This phenomenon was also not seen in assays of leukocytes from carriers for Sandhoff disease, a condition associated with a reduction in the amount of hex B. "

Related Drugs and Biologics

1.	Peptide Hydrolases (Proteases)
2.	Hexosaminidase A (Hex A)
3.	Isoenzymes
4.	Glycoside Hydrolases (Endoglycosidases)
5.	Protein Isoforms (Isoforms)
6.	Glucuronidase
7.	Galactosidases
8.	Mannosidases
9.	alpha-L-Fucosidase (Fucosidase)
10.	beta-N-Acetylhexosaminidases

Related Therapies and Procedures

1.	Oral Administration