Recombinant Mouse Glucosylceramidase (Gba) | MyBioSource MBS955268 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Mouse Glucosylceramidase (Gba)

catalog :

MBS955268

quantity :

0.01 mg (E-Coli)

price :

200 USD

more info or order :

MyBioSource product webpage

image

image 1 :

product information

catalog number :

MBS955268

products type :

Recombinant Protein

products full name :

Recombinant Mouse Glucosylceramidase (Gba)

products short name :

[Glucosylceramidase (Gba)]

products name syn :

[Glucosylceramidase; EC=3.2.1.45; Acid beta-glucosidase; Beta-glucocerebrosidase; D-glucosyl-N-acylsphingosine glucohydrolase]

other names :

[glucosylceramidase; Glucosylceramidase; glucosylceramidase; glucocerebrosidase; acid beta glucosidase; beta-glucocerebrosidase; D-glucosyl-N-acylsphingosine glucohydrolase; glucosidase, beta, acid; Acid beta-glucosidase; Beta-glucocerebrosidase; D-glucosyl-N-acylsphingosine glucohydrolase]

products gene name :

[Gba]

other gene names :

[Gba; Gba; GC; GBA1; GLUC; GCase; betaGC]

uniprot entry name :

GLCM_MOUSE

host :

E Coli or Yeast or Baculovirus or Mammalian Cell

sequence positions :

[20-515aa; Full Length of Mature Protein]

sequence length :

515

sequence :

AQPCIPKSFGYSSVVCVCNASYCDSLDPVTLPALGTFSR
YESTRRGRRMELSVGAIQANRTGTGLLLTLQPEKKFQKV
KGFGGAMTDATALNILALSPPTQKLLLRSYFSTNGIEYN
IIRVPMASCDFSIRVYTYADTPNDFQLSNFSLPEEDTKL
KIPLIHQALKMSSRPISLFASPWTSPTWLKTNGRVNGKG
SLKGQPGDIFHQTWANYFVKFLDAYAKYGLRFWAVTAEN
EPTAGLFTGYPFQCLGFTPEHQRDFISRDLGPALANSSH
DVKLLMLDDQRLLLPRWAEVVLSDPEAAKYVHGIAVHWY
MDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSV
RLGSWDRGMQYSHSIITNLLYHVTGWTDWNLALNPEGGP
NWVRNFVDSPIIVDIPKDAFYKQPMFYHLGHFSKFIPEG
SQRVALVASESTDLETVALLRPDGSAVVVVLNRSSEDVP
LTISDPDLGFLETVSPGYSIHTYLWRRQ

purity :

>=90%

form :

Liquid containing glycerol

storage stability :

Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.

image1 heading :

SDS-Page

other info1 :

Species: Mus musculus (Mouse)

other info2 :

Production Note: Special Offer: The E Coli host-expressed protein is manufactured from a stock plasmid containing the protein gene. E Colihost-expressed protein is stocked in different unit sizes ranging from as small as 10 ug to as large as 1 mg. Bulk inventory is also available. The E Coli host-expressed protein has been ordered over and over again by researchers and has stood the test of time as both a robust protein and important target for the research community. It is part of our new program to make our most popular protein targets and corresponding hosts available in expanded unit sizes and with a quick processing time. Select E Coli host-expressed protein for the fastest delivery among all hosts. Please contact our technical support team or email to support@mybiosource.com for more details.

products categories :

Neuroscience

ncbi gi num :

116734815

ncbi acc num :

NP_001070879.1

ncbi gb acc num :

NM_001077411.2

uniprot acc num :

P17439

ncbi mol weight :

57,622 Da

ncbi pathways :

Glycosphingolipid Metabolism Pathway (926780); Lysosome Pathway (99272); Lysosome Pathway (96865); Metabolism Pathway (926669); Metabolism Of Lipids And Lipoproteins Pathway (926687); Other Glycan Degradation Pathway (83175); Other Glycan Degradation Pathway (346); Sphingolipid Metabolism Pathway (83193); Sphingolipid Metabolism Pathway (926778); Sphingolipid Metabolism Pathway (369)

uniprot summary :

GBA: Defects in GBA are the cause of Gaucher disease (GD); also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Different clinical forms are recognized depending on the presence (neuronopathic forms) or absence of central nervous system involvement, severity and age of onset. Defects in GBA are the cause of Gaucher disease type 1 (GD1); also known as adult non-neuronopathic Gaucher disease. GD1 is characterized by hepatosplenomegaly with consequent anemia and thrombopenia, and bone involvement. The central nervous system is not involved. Defects in GBA are the cause of Gaucher disease type 2 (GD2); also known as acute neuronopathic Gaucher disease. GD2 is the most severe form and is universally progressive and fatal. It manifests soon after birth, with death generally occurring before patients reach two years of age. Defects in GBA are the cause of Gaucher disease type 3 (GD3); also known as subacute neuronopathic Gaucher disease. GD3 has central nervous manifestations. Defects in GBA are the cause of Gaucher disease type 3C (GD3C); also known as pseudo-Gaucher disease or Gaucher-like disease. Defects in GBA are the cause of Gaucher disease perinatal lethal (GDPL). It is a distinct form of Gaucher disease type 2, characterized by fetal onset. Hydrops fetalis, in utero fetal death and neonatal distress are prominent features. When hydrops is absent, neurologic involvement begins in the first week and leads to death within 3 months. Hepatosplenomegaly is a major sign, and is associated with ichthyosis, arthrogryposis, and facial dysmorphism. Perinatal lethal Gaucher disease is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Defects in GBA contribute to susceptibility to Parkinson disease (PARK). A complex neurodegenerative disorder characterized by bradykinesia, resting tremor, muscular rigidity and postural instability. Additional features are characteristic postural abnormalities, dysautonomia, dystonic cramps, and dementia. The pathology of Parkinson disease involves the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (intraneuronal accumulations of aggregated proteins), in surviving neurons in various areas of the brain. The disease is progressive and usually manifests after the age of 50 years, although early-onset cases (before 50 years) are known. The majority of the cases are sporadic suggesting a multifactorial etiology based on environmental and genetic factors. However, some patients present with a positive family history for the disease. Familial forms of the disease usually begin at earlier ages and are associated with atypical clinical features. Belongs to the glycosyl hydrolase 30 family. 3 isoforms of the human protein are produced by alternative splicing. Protein type: Hydrolase; Glycan Metabolism - other glycan degradation; Lipid Metabolism - sphingolipid; EC 3.2.1.45. Cellular Component: lysosomal lumen; membrane; lysosome; lysosomal membrane. Molecular Function: hydrolase activity; hydrolase activity, acting on glycosyl bonds; receptor binding; glucosylceramidase activity. Biological Process: glucosylceramide catabolic process; negative regulation of MAP kinase activity; sphingolipid metabolic process; skin morphogenesis; metabolic process; response to glucocorticoid stimulus; response to testosterone stimulus; positive regulation of protein amino acid dephosphorylation; regulation of water loss via skin; response to estrogen stimulus; carbohydrate metabolic process; negative regulation of interleukin-6 production; sphingosine biosynthetic process; ceramide biosynthetic process; lipid metabolic process; response to pH

size1 :

0.01 mg (E-Coli)

price1 :

200 USD

size2 :

0.05 mg (E-Coli)

price2 :

260

size3 :

0.1 mg (E-Coli)

price3 :

430

size4 :

0.2 mg (E-Coli)

price4 :

685

size5 :

0.5 mg (E-Coli)

price5 :

1125

size6 :

1 mg (E-Coli)

price6 :

1725

more info or order :

MyBioSource product webpage