Version 2.78

24075-4 Beta-N-acetylhexosaminidase [Enzymatic activity/mass] in Leukocytes

Part Descriptions

LP14022-5   Beta-N-acetylhexosaminidase
An enzyme hydrolyzing terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides. It acts on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES. Hexosaminidase A cleaves GM2, GA2, globosides, and hexosamine oligosaccharides. Hexosaminidase B cleaves all the above substrates except GM2. Deficiency of Hexosaminidase A causes TAY-SACHS DISEASE, while deficiency of both A and B isozymes causes SANDHOFF DISEASE. The enzyme has also been used as a tumor marker to distinguish between malignant and benign disease. Source: National Library of Medicine, MeSH 2006

LP14022-5   Beta-N-acetylhexosaminidase
Beta-N-acetylhexosaminidase is also known as hexosaminidase and has two forms, A and B. Hexosaminidase B is used to screen for Tay-Sachs disease. Source: Regenstrief Institute

Fully-Specified Name

Component
Beta-N-acetylhexosaminidase
Property
CCnt
Time
Pt
System
WBC
Scale
Qn
Method

Additional Names

Short Name
B-NAH WBC-cCnt
Display Name
Beta-N-acetylhexosaminidase (WBC) [Catalytic activity/Mass]
Consumer Name Alpha Get Info
Beta-N-acetylhexosaminidase, White blood cells

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 1.0o
Last Updated
Version 2.73
Order vs. Observation
Both
Common Test Rank Get Info
7615

Member of these Panels

LOINC Long Common Name
87544-3 Beta-N-acetylhexosaminidase.A and beta-N-acetylhexosaminidase panel [Enzymatic activity/mass] - Leukocytes
87543-5 Beta-N-acetylhexosaminidase.A and beta-N-acetylhexosaminidase with reflex to HEXA gene panel [Enzymatic activity/mass] - Leukocytes

Language Variants Get Info

Tag Language Translation
es-MX Spanish (Mexico) Beta-N-acetilhexosaminidasa:Contenido catalítico:Punto temporal:células blancas de la sangre:Cuantitativo:
es-ES Spanish (Spain) Beta-N-acetilhexosaminidasa:Contenido catalítico:Punto temporal:Células sanguineas blancas:Qn:
Synonyms: Cuantitativo
es-AR Spanish (Argentina) beta-N-acetilhexosaminidasa:contenido catalítico:punto en el tiempo:leucocito:cuantitativo:
et-EE Estonian (Estonia) Beeta-N-atsetüülheksosaminidaas:CCnt:Pt:WBC:Qn:
Synonyms: Juhuslik Kvantitatiivne Leukotsüüdid
fr-BE French (Belgium) Béta-N-acétylhexosaminidase:Contenu catalytique:Temps ponctuel:Leucocytes:Quantitatif:
fr-FR French (France) Bêta N-hexosaminidase:Catalytique/Masse:Ponctuel:Leucocytes:Numérique:
fr-CA French (Canada) Bêta-N-acétylhexosaminidase:Contenu catalytique:Temps ponctuel:Leucocytes:Quantitatif:
it-IT Italian (Italy) Beta-N-acetilesosaminidasi:CCnt:Pt:WBC:Qn:
Synonyms: Chimica Contenuto Catalitico Globuli bianchi Punto nel tempo (episodio)
ko-KR Korean (Korea, Republic Of) 베타-N-아세틸헥소스아미니다아제:촉매구성:검사시점:백혈구:정량:
nl-NL Dutch (Netherlands) bèta-N-acetylhexosaminidase:enzymatische activiteit/massa:moment:leukocyten:kwantitatief:
Synonyms: Beta-N-acetylhexosaminidase witte bloedcellen
pt-BR Portuguese (Brazil) Beta N-acetilhexosaminidase:CCnt:Pt:Leucoc:Qn:
Synonyms: ; B-NAH; GM2-gangliosidosis; Hexosaminidase; Catalytic Content; Point in time; Random; Leukocytes; White blood cells; Leucocs; Quantitative; QNT; Quant; Quan; B; Chemistry
ru-RU Russian (Russian Federation) Бета-N-ацетилгексозаминидаза:КаталСод:ТчкВрм:Лейкоциты:Колич:
Synonyms: Каталитическое содержание Количественный Точка во времени;Момент
tr-TR Turkish (Turkey) Beta-N-asetilhekzosaminidaz:Ktlİçrk:Zmlı:Lökosit:Kant:
Synonyms: WBC
zh-CN Chinese (China) Beta-N-乙酰己糖胺酶:催化含量:时间点:白细胞:定量型:
Synonyms: B Beta-N-乙酰氨基己糖苷酶;B-NAH;GM2-神经节苷脂沉积症;GM2-神经节苷脂贮积病;β-N-乙酰己糖胺酶;β-N-乙酰氨基己糖苷酶;己糖胺酶;氨基己糖苷酶 N 型 WBC;WBCs;白血球;白血细胞 β 催化含量(单位质量);单位质量的酶单位数 化学;化学检验项目;化学检验项目类;化学类;化学试验;非刺激耐受型化学检验项目;非刺激耐受型化学检验项目类;非刺激耐受型化学试验;非刺激耐受型化学试验类 可用数量表示的;定量性;数值型;数量型;连续数值型标尺 时刻;随机;随意;瞬间 贝塔

Example Units

Unit Source
nmol/h/mg{protein} Example UCUM Units
nmol/mg prot/H REGENSTRIEF

50759-0 Beta-N-acetylhexosaminidase.A [Enzymatic activity/mass] in Leukocytes

Part Descriptions

LP14296-5   Beta-N-acetylhexosaminidase.A
Beta-hexosaminidase subunit alpha (HEXA) is responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.[UniProt: P06965] Reduced or absent levels of HEXA and accumulation of GM2 gangliosides in the neuronal cells is characteristic of GM2-gangliosidosis 1 (GM2G1), and autosomal recessive lysosomal storage disease that leads to neurodegeneration and, in the infantile form, death in early childhood.[OMIM: 272800] Source: Regenstrief LOINC

Fully-Specified Name

Component
Beta-N-acetylhexosaminidase.A
Property
CCnt
Time
Pt
System
WBC
Scale
Qn
Method

Additional Names

Short Name
Hexosaminidase A WBC-cCnt
Display Name
Beta-N-acetylhexosaminidase.A (WBC) [Catalytic activity/Mass]
Consumer Name Alpha Get Info
Beta-N-acetylhexosaminidase.A, White blood cells

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 2.22
Last Updated
Version 2.42
Order vs. Observation
Both

Member of these Panels

LOINC Long Common Name
87544-3 Beta-N-acetylhexosaminidase.A and beta-N-acetylhexosaminidase panel [Enzymatic activity/mass] - Leukocytes
87543-5 Beta-N-acetylhexosaminidase.A and beta-N-acetylhexosaminidase with reflex to HEXA gene panel [Enzymatic activity/mass] - Leukocytes

Language Variants Get Info

Tag Language Translation
de-AT German (Austria) Synonyms: Beta-Hexosaminidase /Leukozyten
es-MX Spanish (Mexico) Beta-N-acetilhexosaminidasa.A:Contenido catalítico:Punto temporal:células blancas de la sangre:Cuantitativo:
es-ES Spanish (Spain) Beta-N-acetilhexosaminidasa.A:Contenido catalítico:Punto temporal:Células sanguineas blancas:Qn:
Synonyms: Cuantitativo
et-EE Estonian (Estonia) Beeta-N-atsetüülheksosaminidaas.A:CCnt:Pt:WBC:Qn:
Synonyms: Juhuslik Kvantitatiivne Leukotsüüdid
fr-FR French (France) Bêta N-hexosaminidase A:Catalytique/Masse:Ponctuel:Leucocytes:Numérique:
fr-CA French (Canada) Bêta-N-acétylhexosaminidase.A:Contenu catalytique:Temps ponctuel:Leucocytes:Quantitatif:
fr-BE French (Belgium) Béta-N-acétylhexosaminidase.A:Contenu catalytique:Temps ponctuel:Leucocytes:Quantitatif:
it-IT Italian (Italy) Beta-N-acetilesosaminidasi.A:CCnt:Pt:WBC:Qn:
Synonyms: Chimica Contenuto Catalitico Globuli bianchi Punto nel tempo (episodio)
nl-NL Dutch (Netherlands) bèta-N-acetylhexosaminidase.A:enzymatische activiteit/massa:moment:leukocyten:kwantitatief:
Synonyms: Beta-N-acetylhexosaminidase Beta-N-acetylhexosaminidase.A witte bloedcellen
pt-BR Portuguese (Brazil) Beta-N-acetilhexosaminidase A:CCnt:Pt:Leucoc:Qn:
Synonyms: ; Hexosaminidase A; Sandoff disease; Hex A; B-NAH; GM2-gangliosidosis; Catalytic Content; Point in time; Random; Leukocytes; White blood cells; Leucocs; Quantitative; QNT; Quant; Quan; B; Chemistry
ru-RU Russian (Russian Federation) Бета-N-Ацетилгексозаминидаза.А:КаталСод:ТчкВрм:Лейкоциты:Колич:
Synonyms: Каталитическое содержание Количественный Точка во времени;Момент
tr-TR Turkish (Turkey) Beta-N-asetilhekzosaminidaz.A:Ktlİçrk:Zmlı:Lökosit:Kant:
Synonyms: WBC
zh-CN Chinese (China) Beta-N-乙酰己糖胺酶.A:催化含量:时间点:白细胞:定量型:
Synonyms: A 型 B Beta-N-乙酰己糖胺酶 A;Beta-N-乙酰氨基己糖苷酶 A;Beta-N-乙酰氨基己糖苷酶.A;Hex A;Sandoff 氏病;Sandoff 病;β-N-乙酰己糖胺酶 A;β-N-乙酰己糖胺酶.A;β-N-乙酰氨基己糖苷酶 A;β-N-乙酰氨基己糖苷酶.A;己糖胺酶 A;己糖胺酶.A;氨基己糖苷酶 A;氨基己糖苷酶.A Beta-N-乙酰氨基己糖苷酶;B-NAH;GM2-神经节苷脂沉积症;GM2-神经节苷脂贮积病;β-N-乙酰己糖胺酶;β-N-乙酰氨基己糖苷酶;己糖胺酶;氨基己糖苷酶 N 型 WBC;WBCs;白血球;白血细胞 β 催化含量(单位质量);单位质量的酶单位数 化学;化学检验项目;化学检验项目类;化学类;化学试验;非刺激耐受型化学检验项目;非刺激耐受型化学检验项目类;非刺激耐受型化学试验;非刺激耐受型化学试验类 可用数量表示的;定量性;数值型;数量型;连续数值型标尺 时刻;随机;随意;瞬间 贝塔

Example Units

Unit Source
nmol/h/mg{protein} Example UCUM Units

23825-3 Beta-N-acetylhexosaminidase.A/Beta-n-acetylhexosaminidase.total in Leukocytes

Part Descriptions

LP14296-5   Beta-N-acetylhexosaminidase.A
Beta-hexosaminidase subunit alpha (HEXA) is responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.[UniProt: P06965] Reduced or absent levels of HEXA and accumulation of GM2 gangliosides in the neuronal cells is characteristic of GM2-gangliosidosis 1 (GM2G1), and autosomal recessive lysosomal storage disease that leads to neurodegeneration and, in the infantile form, death in early childhood.[OMIM: 272800] Source: Regenstrief LOINC

Fully-Specified Name

Component
Beta-N-acetylhexosaminidase.A/Beta-N-acetylhexosaminidase.total
Property
CFr
Time
Pt
System
WBC
Scale
Qn
Method

Additional Names

Short Name
Hexosaminidase A CFr WBC
Display Name
Beta-N-acetylhexosaminidase.A/Beta-n-acetylhexosaminidase.total (WBC) [Catalytic fraction]
Consumer Name Alpha Get Info
Beta-N-acetylhexosaminidase.A/Beta-n-acetylhexosaminidase.total, White blood cells

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 1.0n
Last Updated
Version 2.73
Order vs. Observation
Observation
Common Test Rank Get Info
7850

Member of these Panels

LOINC Long Common Name
87544-3 Beta-N-acetylhexosaminidase.A and beta-N-acetylhexosaminidase panel [Enzymatic activity/mass] - Leukocytes
87543-5 Beta-N-acetylhexosaminidase.A and beta-N-acetylhexosaminidase with reflex to HEXA gene panel [Enzymatic activity/mass] - Leukocytes

Language Variants Get Info

Tag Language Translation
es-AR Spanish (Argentina) beta-N-acetilhexosaminidasa.A/beta-N-acetilhexosaminidasa.total:fracción catalítica:punto en el tiempo:leucocito:cuantitativo:
es-MX Spanish (Mexico) Beta-N-acetilhexosaminidasa.A / Beta-N-acetilhexosaminidasa.total:Fracción catalítica:Punto temporal:células blancas de la sangre:Cuantitativo:
es-ES Spanish (Spain) Beta-N-acetilhexosaminidasa.A/Beta-n-acetilhexosaminidasa.total:Fracción Catalítica:Punto temporal:Células sanguineas blancas:Qn:
Synonyms: Cuantitativo
et-EE Estonian (Estonia) Beeta-N-atsetüülheksosaminidaas.A/beeta-n-atsetüülheksosaminidaas.üld:CFr:Pt:WBC:Qn:
Synonyms: Juhuslik Kvantitatiivne Leukotsüüdid
fr-FR French (France) Bêta N-hexosaminidase A/Bêta N-hexosaminidases totales:Fraction catalytique:Ponctuel:Leucocytes:Numérique:
fr-CA French (Canada) Bêta-N-acétylhexosaminidase.A/Bêta-N-acétylhexosaminidase.totale:Fraction catalytique:Temps ponctuel:Leucocytes:Quantitatif:
fr-BE French (Belgium) Béta-N-acétylhexosaminidase.A/Bèta-N-acétylhexosaminidase.totale:Fraction catalytique:Temps ponctuel:Leucocytes:Quantitatif:
it-IT Italian (Italy) Beta-N-acetilesosaminidasi.A/Beta-N-acetilesosaminidasi.totale:CFr:Pt:WBC:Qn:
Synonyms: Chimica Frazione Catalitica Globuli bianchi Punto nel tempo (episodio)
ko-KR Korean (Korea, Republic Of) 베타-N-아세틸헥소스아미니다아제.A/총베타-N-아세틸헥소스아미니다아제:촉매분획:검사시점:백혈구:정량:
nl-NL Dutch (Netherlands) bèta-N-acetylhexosaminidase.A/bèta-n-acetylhexoaminidase.totaal:enzymatische fractie:moment:leukocyten:kwantitatief:
Synonyms: Beta-N-acetylhexosaminidase Beta-N-acetylhexosaminidase.A witte bloedcellen
pt-BR Portuguese (Brazil) Beta-N-acetilhexosaminidase.A/Beta-N-acetilhexosaminidase.total:CFr:Pt:Leucoc:Qn:
Synonyms: ; Hexosaminidase A; Sandoff disease; Hex A; B-NAH; GM2-gangliosidosis; Fr; Catalytic Fraction; Percent; Point in time; Random; Leukocytes; White blood cells; Leucocs; Quantitative; QNT; Quant; Quan; Tot; Totl; B; Chemistry
ru-RU Russian (Russian Federation) Бета-N-Ацетилгексозаминидаза.А/Бета-n-ацетилгексозаминидаза.общий:КаталФрак:ТчкВрм:Лейкоциты:Колич:
Synonyms: Каталитическая фракция Количественный Точка во времени;Момент
tr-TR Turkish (Turkey) Beta-N-asetilhekzosaminidaz.A/Beta-n-asetilhekzosaminidaz.total:KtlFr:Zmlı:Lökosit:Kant:
Synonyms: WBC
uk-UA Ukrainian (Ukraine) Бета-N-ацетилгексозамінідаза.А/Бета-N-ацетилгексозамінідаза.загальна:Каталітична фракція:МоментЧасу:Білі кров'яні клітини:Кількісно:
Synonyms: B; Beta-hexosaminidase subunit alpha; B-NAH; Catalytic Fraction; Chemistry; GM2 Gangliosidoses; GM2-gangliosidosis; Hex A; Hex B; HEXA; Hexosaminidase; Hexosaminidase A; Leukocytes; Percent; Point in time; QNT; Quan; Quant; Quantitative; Random; Sandhoff disease; Tay Sachs Disease; Tot; Totl; WBCs; White blood cells
zh-CN Chinese (China) Beta-N-乙酰己糖胺酶.A/Beta-n-乙酰氨基已糖苷酶.总计:催化分数:时间点:白细胞:定量型:
Synonyms: A 型 B Beta-N-乙酰己糖胺酶 A;Beta-N-乙酰氨基己糖苷酶 A;Beta-N-乙酰氨基己糖苷酶.A;Hex A;Sandoff 氏病;Sandoff 病;β-N-乙酰己糖胺酶 A;β-N-乙酰己糖胺酶.A;β-N-乙酰氨基己糖苷酶 A;β-N-乙酰氨基己糖苷酶.A;己糖胺酶 A;己糖胺酶.A;氨基己糖苷酶 A;氨基己糖苷酶.A Beta-N-乙酰氨基己糖苷酶;B-NAH;GM2-神经节苷脂沉积症;GM2-神经节苷脂贮积病;β-N-乙酰己糖胺酶;β-N-乙酰氨基己糖苷酶;己糖胺酶;氨基己糖苷酶 Beta-n-乙酰氨基已糖苷酶（Beta-N-乙酰氨基己糖苷酶、B-NAH、β-N-乙酰己糖胺酶、β-N-乙酰氨基己糖苷酶、己糖胺酶、氨基己糖苷酶）.总计;Beta-n-乙酰己糖胺酶总计 N 型 WBC;WBCs;白血球;白血细胞 β 全体数量 全部 共计 化学;化学检验项目;化学检验项目类;化学类;化学试验;非刺激耐受型化学检验项目;非刺激耐受型化学检验项目类;非刺激耐受型化学试验;非刺激耐受型化学试验类 可用数量表示的;定量性;数值型;数量型;连续数值型标尺 总 总和 总数 总量 总额 时刻;随机;随意;瞬间 汇总 贝塔

Example Units

Unit Source
% Example UCUM Units
% REGENSTRIEF