07 Glycogen
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- muscles cannot release glucose from glycogen
![](https://photos.thisispiggy.com/file/wikiFiles/21F7904F-6760-4B46-AE06-75D981EF0FB6.jpg)
- 1-4 linear linkage
- 1-6 branching
- branching makes it more soluble and better storage
![](https://photos.thisispiggy.com/file/wikiFiles/349B5EE0-C4CF-4A60-8D7E-DFF3210129F8.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/78B627E6-F5AC-4176-81A4-5B3D3C27F06D.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/87D1C4BE-1FBD-426C-8727-D6D8235F839B.jpg)
- lysosomes can collect glycogen and break it down to glucose
![](https://photos.thisispiggy.com/file/wikiFiles/6B48805D-84F7-45D3-BE96-59A72A86C0D3.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/7767CA9A-AA7A-4BF4-AB68-B7D426D3FD73.jpg)
- glycogen phosphorylase shrink purple link down to 3 or so
- debranching enzyme moves purple to red, releasing one molecule of glucose (green)
![](https://photos.thisispiggy.com/file/wikiFiles/955E285E-3A4A-405A-81BA-7CB4D25771ED.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/879765D4-751C-4F4E-ADED-2FB3F3F0FED7.jpg)
- glucagon/E phosphorylates both enzymes both have opposite effects
- glycogen phosphorylase activity increases
- glycogen synthase activity decreases
![](https://photos.thisispiggy.com/file/wikiFiles/EB47750B-21DF-4949-8DC5-B06B8E73B16A.jpg)
- insulin results in dephosphorylation of both and opposite effects
![](https://photos.thisispiggy.com/file/wikiFiles/2C1D9007-E086-42F3-A22D-FC92BD97E945.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/04A791BA-4A49-4B47-A368-E3DB2316EC87.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/1C364FD6-F6ED-4FF0-BD5D-D5D8869199A8.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/E90AE91B-191B-4E81-B2CE-1F02F8C2B762.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/2D408538-A47A-44EC-8F7C-369CEDA285A3.jpg)
- activate glycogen phosphorylase in absence of hormones
- immediate activation instead of hormonal
![](https://photos.thisispiggy.com/file/wikiFiles/5C64854D-2B6C-4AA6-838C-7BD02645ACB4.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/98A874D5-FA51-4F6F-8CED-F33C24F29970.jpg)
Glycogen Storage Diseases
![](https://photos.thisispiggy.com/file/wikiFiles/A3DD0BA9-366C-4431-A1F3-76523DC31D2E.jpg)
- liver defective enzyme: hypoglycemia
- enzyme defective in muscle: weakness
Von Gierke
![](https://photos.thisispiggy.com/file/wikiFiles/0EA68298-ED04-4831-806B-75261E490B0F.jpg)
- can't release glucose between meals
- can't get glucose out of liver = hepatomegaly
![](https://photos.thisispiggy.com/file/wikiFiles/D4824D28-5B6C-408B-8005-43DC466D632A.jpg)
- muscles will be ok
- no symptoms of muscle weakness
![](https://photos.thisispiggy.com/file/wikiFiles/07177D7D-9791-4E3C-9122-A44CA9E348D1.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/677B3BAA-1457-4A0E-94B0-91DD9C736FDD.jpg)
- consume corn starch between meals
Pompe
![](https://photos.thisispiggy.com/file/wikiFiles/9805E919-3583-41D1-82F9-A731DE0A53AB.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/E2E862FA-D74A-40E0-9396-00CB03D41250.jpg)
- liver is ok, continue make glucose
- no hypoglycemia symptoms
![](https://photos.thisispiggy.com/file/wikiFiles/98949B83-AFD0-4BC4-9F5B-E082BD02EF13.jpg)
- entrapment of glycogen inside muscle cells
- enlarged tongue classic finding
Cori
![](https://photos.thisispiggy.com/file/wikiFiles/3C8384E4-276C-4048-A6DD-4CC11E07F0FE.jpg)
![](https://photos.thisispiggy.com/file/wikiFiles/24353400-09E3-46BC-A441-B9CF5C45A411.jpg)
- gluconeogenesis intact from other sources: hypoglycemia not as severe
- no glucose in muscles either: weakness
![](https://photos.thisispiggy.com/file/wikiFiles/5F95A878-1E57-46B5-BCEE-D984D8E1DBFF.jpg)
- usually no seizures
- muscle symptoms distinguish from Von Gierke
McArdle
![](https://photos.thisispiggy.com/file/wikiFiles/99C3F9B3-6519-46B8-B5F9-3786C23C0968.jpg)
- no liver problems, no hypoglycemis
- muscle difficulties
- can get muscle damage with exercise
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