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Mouse Anti-UCHL1 Recombinant Antibody (BH7) (CBMAB-0921CQ)

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Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat, Cattle
Clone
BH7
Antibody Isotype
IgG1
Application
IF, WB

Basic Information

Immunogen
Recombinant full length human PGP9.5
Specificity
Human, Mouse, Rat, Cattle
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Ubiquitin C-Terminal Hydrolase L1
Introduction
The protein encoded by this gene belongs to the peptidase C12 family. Ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins. This enzyme is a thiol protease that recognizes and hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. Also binds to free monoubiquitin and may prevent its degradation in lysosomes. The homodimer may have ATP-independent ubiquitin ligase activity.
Entrez Gene ID
Human7345
Mouse22223
Rat29545
Cattle514394
UniProt ID
HumanP09936
MouseQ9R0P9
RatQ00981
CattleP23356
Alternative Names
NDGOA; PARK5; PGP95; PGP9.5; Uch-L1; HEL-117; PGP 9.5; HEL-S-53
Function
Ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins (Probable). This enzyme is a thiol protease that recognizes and hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin (PubMed:9774100, PubMed:8639624, PubMed:12408865, PubMed:23359680).
Also binds to free monoubiquitin and may prevent its degradation in lysosomes (By similarity).
The homodimer may have ATP-independent ubiquitin ligase activity (PubMed:12408865).
Biological Process
Biological Process adult walking behavior Source:Ensembl
Biological Process axon target recognition Source:Ensembl
Biological Process axonal transport of mitochondrion Source:Ensembl
Biological Process cellular response to xenobiotic stimulus Source:Ensembl
Biological Process eating behavior Source:Ensembl
Biological Process male germ cell proliferation Source:Ensembl
Biological Process muscle cell development Source:Ensembl
Biological Process negative regulation of MAP kinase activity Source:BHF-UCL1 Publication
Biological Process neuromuscular process Source:Ensembl
Biological Process proteasome-mediated ubiquitin-dependent protein catabolic process Source:ParkinsonsUK-UCL1 Publication
Biological Process protein deubiquitination Source:UniProtKB1 Publication
Biological Process regulation of macroautophagy Source:ParkinsonsUK-UCL1 Publication
Biological Process response to ischemia Source:Ensembl
Biological Process sensory perception of pain Source:Ensembl
Cellular Location
Cytoplasm
Endoplasmic reticulum membrane
About 30% of total UCHL1 is associated with membranes in brain.
Involvement in disease
Parkinson disease 5 (PARK5):
A complex neurodegenerative disorder with manifestations ranging from typical Parkinson disease to dementia with Lewy bodies. Clinical features include parkinsonian symptoms (resting tremor, rigidity, postural instability and bradykinesia), dementia, diffuse Lewy body pathology, autonomic dysfunction, hallucinations and paranoia.
Spastic paraplegia 79, autosomal recessive (SPG79):
A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG79 is characterized by childhood onset blindness, cerebellar ataxia, nystagmus, dorsal column dysfunction, and spasticity with upper motor neuron dysfunction.
PTM
O-glycosylated.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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