Anti-MANBA Antibody (CAB15289)
- SKU:
- CAB15289
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-MANBA Antibody |
抗体コード: | CAB15289 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC |
反応性: | Human, Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 18-300 of human MANBA (NP_005899.3). |
申し込み: | WB IHC |
推奨希釈: | WB 1:200 - 1:2000 IHC 1:50 - 1:200 |
反応性: | Human, Mouse |
ポジティブサンプル: | Mouse lung, Mouse testis |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 18-300 of human MANBA (NP_005899.3). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | AAEL SYSL RGNW SICN GNGS LELP GAVP GCVH SALF QQGL IQDS YYRF NDLN YRWV SLDN WTYS KEFK IPFE ISKW QKVN LILE GVDT VSKI LFNE VTIG ETDN MFNR YSFD ITNV VRDV NSIE LRFQ SAVL YAAQ QSKA HTRY QVPP DCPP LVQK GECH VNFV RKEQ CSFS WDWG PSFP TQGI WKDV RIEA YNIC HLNY FTFS PIYD KSAQ EWNL EIES TFDV VSSK PVGG QVIV AIPK LQTQ QTYS IELQ PGKR IVEL FVNI SKNI TVET WWPH GHGN QTG |
遺伝子ID: | 4126 |
Uniprot: | O00462 |
セルラーロケーション: | Lysosome |
計算された分子量: | 100kDa |
観察された分子量: | 101kDa |
同義語: | MANBA, MANB1 |
バックグラウンド: | This gene encodes a member of the glycosyl hydrolase 2 family. The encoded protein localizes to the lysosome where it is the final exoglycosidase in the pathway for N-linked glycoprotein oligosaccharide catabolism. Mutations in this gene are associated with beta-mannosidosis, a lysosomal storage disease that has a wide spectrum of neurological involvement. |
UniProt Protein Function: | MANBA: Exoglycosidase that cleaves the single beta-linked mannose residue from the non-reducing end of all N-linked glycoprotein oligosaccharides. Defects in MANBA are the cause of a mild disorder that affects peripheral and central nervous system myelin. Defects in MANBA are the cause of lysosomal beta- mannosidosis (LYSBMAN). Beta-mannosidosis is an autosomal recessive lysosomal storage disease of glycoprotein catabolism. Clinical features are heterogeneous with a wide range of symptoms and age of onset. The disease is associated with a range of neurological involvement, including various degrees of mental retardation in most of the cases, hearing loss and speech impairment, hypotonia, epilepsy and peripheral neuropathy. Affected individuals have a profound reduction in beta-mannosidase activity in plasma, fibroblasts and leukocytes. Belongs to the glycosyl hydrolase 2 family. |
UniProt Protein Details: | Protein type:EC 3.2.1.25; Glycan Metabolism - other glycan degradation; Hydrolase Chromosomal Location of Human Ortholog: 4q24 Cellular Component: intracellular membrane-bound organelle; lysosomal lumen Molecular Function:beta-mannosidase activity Biological Process: carbohydrate metabolic process; oligosaccharide catabolic process Disease: Mannosidosis, Beta A, Lysosomal |
NCBI Summary: | This gene encodes a member of the glycosyl hydrolase 2 family. The encoded protein localizes to the lysosome where it is the final exoglycosidase in the pathway for N-linked glycoprotein oligosaccharide catabolism. Mutations in this gene are associated with beta-mannosidosis, a lysosomal storage disease that has a wide spectrum of neurological involvement. [provided by RefSeq, Jul 2008] |
UniProt Code: | O00462 |
NCBI GenInfo Identifier: | 51338734 |
NCBI Gene ID: | 4126 |
NCBI Accession: | O00462.3 |
UniProt Secondary Accession: | O00462,Q96BC3, Q9NYX9, |
UniProt Related Accession: | O00462 |
Molecular Weight: | 100,895 Da |
NCBI Full Name: | Beta-mannosidase |
NCBI Synonym Full Names: | mannosidase beta |
NCBI Official Symbol: | MANBA |
NCBI Official Synonym Symbols: | MANB1 |
NCBI Protein Information: | beta-mannosidase |
UniProt Protein Name: | Beta-mannosidase |
UniProt Synonym Protein Names: | Lysosomal beta A mannosidase; Mannanase; Mannase |
Protein Family: | Beta-mannosidase |
UniProt Gene Name: | MANBA |
UniProt Entry Name: | MANBA_HUMAN |
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